105ContextIsovaleric AciduriaIsovaleric acidemia (IVA) is caused by mutation in the isovaleryl CoA dehydrogenase gene. Isovaleryl CoA dehydrogenase is part of the acyl-CoA dehydrogenase family and is involved in the catabolism of leucine. A defect in this enzyme causes accumulation of ammonia, ketone bodies, Isovaleryl/2-Methylbutyrylcarnitine (C5) in blood; carnitine in plasma; creatinine, and glucose in serum; 3-Hydroxybutyric acid, 3-Hydroxyisovaleric acid, 4-Hydroxyvaleric acid, acetyltryptophan, glycine, acylcarnitin, isovalerylasparagine, isovalerylglycine, isovaleryllysine, isovalerylhistidine and isovaleryltryptophan in urine. Symptoms include encephalopathy, ketosis, metabolic acidosis, pancreatitis, sweaty feet odor, and thrombocytopenia.DiseasePW000091CenterPathwayVisualizationContext9158005050#000099PathwayVisualization6186Valine, Leucine, and Isoleucine DegradationValine, isoleuciine, and leucine are essential amino acids and are identified as the branched-chain amino acids (BCAAs). The catabolism of all three amino acids starts in muscle and yields NADH and FADH2 which can be utilized for ATP generation. The catabolism of all three of these amino acids uses the same enzymes in the first two steps. The first step in each case is a transamination using a single BCAA aminotransferase, with α-ketoglutarate as the amine acceptor. As a result, three different α-keto acids are produced and are oxidized using a common branched-chain α-keto acid dehydrogenase (BCKD), yielding the three different CoA derivatives. Isovaleryl-CoA is produced from leucine by these two reactions, alpha-methylbutyryl-CoA from isoleucine, and isobutyryl-CoA from valine. These acyl-CoA’s undergo dehydrogenation, catalyzed by three different but related enzymes, and the breakdown pathways then diverge. Leucine is ultimately converted into acetyl-CoA and acetoacetate; isoleucine into acetyl-CoA and succinyl-CoA; and valine into propionyl-CoA (and subsequently succinyl-CoA). Under fasting conditions, substantial amounts of all three amino acids are generated by protein breakdown. In muscle, the final products of leucine, isoleucine, and valine catabolism can be fully oxidized via the citric acid cycle; in the liver, they can be directed toward the synthesis of ketone bodies (acetoacetate and acetyl-CoA) and glucose (succinyl-CoA). Because isoleucine catabolism terminates with the production of acetyl-CoA and propionyl-CoA, it is both glucogenic and ketogenic. Because leucine gives rise to acetyl-CoA and acetoacetyl-CoA, it is classified as strictly ketogenic. Metabolic1759100SubPathway12541455Compound37632SubPathway1260940Compound3767101SubPathway1266988Compound37722SubPathway1275940Compound31276808Compound379Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.86Pathway80Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.86Pathway27963521104317Wanders RJ, Duran M, Loupatty FJ: Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway. J Inherit Metab Dis. 2012 Jan;35(1):5-12. doi: 10.1007/s10545-010-9236-x. Epub 2010 Nov 23.86Pathway1CellCL:00000005HepatocyteCL:00001823NeuronCL:000054012AstrocyteCL:00001276MyocyteCL:00001874CardiomyocyteCL:00007467Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:000063911Colorectal Cancer CellCL:00010641Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote18Saccharomyces cerevisiae4932EukaryoteYeast23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat4Arabidopsis thaliana3702EukaryoteThale cress2Bacteria2ProkaryoteBacteria24Solanum lycopersicum4081EukaryoteTomato21Xenopus laevis8355EukaryoteAfrican clawed frog60Nitzschia sp.0001EukaryoteNitzschia410Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote202Spathaspora passalidarum340170EukaryoteSpathaspora passalidarum49Bathymodiolus platifrons220390EukaryoteDeep sea mussel29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast135Felinus9685EukaryoteCat240Plasmodium falciparums121Eukaryote55Yarrowia lipolytica4952Eukaryoteyli196Homo1924EukaryoteHuman230Ambystoma mexicanum8296Eukaryoteaxolotl1CytosolGO:000582911Extracellular SpaceGO:000561531Periplasmic SpaceGO:00056203Mitochondrial MatrixGO:00057592MitochondrionGO:00057395CytoplasmGO:00057374PeroxisomeGO:000577735ChloroplastGO:00095077Endoplasmic Reticulum MembraneGO:000578925Golgi ApparatusGO:000579412Mitochondrial Inner MembraneGO:00057436LysosomeGO:000576413Endoplasmic ReticulumGO:000578316Lysosomal LumenGO:004320214Mitochondrial Outer MembraneGO:000574124Mitochondrial Intermembrane SpaceGO:000575810Cell MembraneGO:000588636MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032532Inner MembraneGO:007025820Endoplasmic Reticulum LumenGO:000578839Mitochondrial membraneGO:00319668Smooth Endoplasmic Reticulum GO:000579027Peroxisome MembraneGO:000577818Melanosome MembraneGO:003316221SynapseGO:004520215NucleusGO:000563440PeriplasmGO:004259719Sarcoplasmic ReticulumGO:00165291LiverBTO:000075972928StomachBTO:0001307155268Blood VesselBTO:0001102741124BrainBTO:000014289169MuscleBTO:0000887141184Adrenal MedullaBTO:00000497183Sympathetic Nervous SystemBTO:000183225IntestineBTO:00006487Nervous SystemBTO:000148411HeartBTO:000056273106KidneyBTO:00006717182Endothelium 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171PW_BS0001154701517PW_BS00011548515101PW_BS0001154991510PW_BS0001155161561PW_BS000115517156PW_BS000115471914PW_BS00004731323PW_BS000024241529PW_BS00002425715291PW_BS000024326812PW_BS00002841685PW_BS000115452817PW_BS000115488810PW_BS00011550586PW_BS00011570231351PW_BS000512102032401PW_BS0005777132210111PW_BS00051211812551PW_BS000588100811961PW_BS00057795611211PW_BS000563979132301PW_BS0005697851521PW_BS000522540L-LeucineHMDB0000687Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. 'BCAA' denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are useful because they are metabolized primarily by muscle. Stress state- e.g surgery, trauma, cirrhosis, infections, fever and starvation--require proportionately more BCAA than other amino acids and probably proportionately more leucine than either valine or isoleucine. BCAA and other amino acids are frequently fed intravenously (TPN) to malnourished surgical patients and in some cases of severe trauma. BCAA, particularly leucine, stimulate protein synthesis, increase reutilization of amino acids in many organs and reduce protein breakdown. Furthermore, leucine can be an important source of calories, and is superior as fuel to the ubiquitous intravenous glucose (dextrose). Leucine also stimulates insulin release, which in turn stimulates protein synthesis and inhibits protein breakdown. These effects are particularly useful in athletic training. BCAA should also replace the use of steroids as commonly used by weightlifters. Huntington's chorea and anorexic disorders both are characterized by low serum BCAA. These diseases, as well as forms of Parkinson's, may respond to BCAA therapy. BCAA, and particularly leucine, are among the amino acids most essential for muscle health. (http://www.dcnutrition.com).61-90-5C00123610615603LEU5880DB00149CC(C)C[C@H](N)C(O)=OC6H13NO2InChI=1S/C6H13NO2/c1-4(2)3-5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1ROHFNLRQFUQHCH-YFKPBYRVSA-N(2S)-2-amino-4-methylpentanoic acid131.1729131.094628665-0.272L-leucine00FDB001946(2s)-2-amino-4-methylpentanoate;(2s)-2-amino-4-methylpentanoic acid;(s)-(+)-leucine;(s)-2-amino-4-methylpentanoate;(s)-2-amino-4-methylpentanoic acid;(s)-2-amino-4-methylvalerate;(s)-2-amino-4-methylvaleric acid;(s)-leucine;4-methyl-l-norvaline;L-(+)-leucine;L-a-aminoisocaproate;L-a-aminoisocaproic acid;L-alpha-aminoisocaproate;L-alpha-aminoisocaproic acid;Leu;Leucine;(2s)-alpha-2-amino-4-methylvaleric acid;(2s)-alpha-leucine;2-amino-4-methylvaleric acid;L;L-leucin;L-leuzin;(2s)-a-2-amino-4-methylvalerate;(2s)-a-2-amino-4-methylvaleric acid;(2s)-alpha-2-amino-4-methylvalerate;(2s)-α-2-amino-4-methylvalerate;(2s)-α-2-amino-4-methylvaleric acid;(2s)-a-leucine;(2s)-α-leucinePW_C000540Leu1582250431556461075647108684816671451887146187425393154255731879181132121544124124102118547KetoleucineHMDB0000695Ketoleucine is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. Ketoleucine is both a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of ketoleucine are associated with maple syrup urine disease (MSUD). MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465).816-66-0C0023370484302K-4CH3-PENTANOATE69DB03229CC(C)CC(=O)C(O)=OC6H10O3InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)BKAJNAXTPSGJCU-UHFFFAOYSA-N4-methyl-2-oxopentanoic acid130.1418130.062994186-1.281ketoisocaproate0-1FDB0126072-keto-4-methylvalerate;2-keto-4-methylvaleric acid;2-ketoisocaproate;2-ketoisocaproic acid;2-oxo-4-methylpentanoate;2-oxo-4-methylpentanoic acid;2-oxo-4-methylvalerate;2-oxo-4-methylvaleric acid;2-oxoisocaproate;2-oxoisocaproic acid;2-oxoleucine;4-methyl-2-oxo-valerate;4-methyl-2-oxo-valeric acid;4-methyl-2-oxopentanoate;4-methyl-2-oxopentanoic acid;Ketoisocaproate;Ketoisocaproic acid;Methyloxovalerate;Methyloxovaleric acid;Oxoisocaproate;Oxoisocaproic acid;A-ketoisocaproate;A-ketoisocaproic acid;A-ketoisocapronate;A-ketoisocapronic acid;A-oxoisocaproate;A-oxoisocaproic acid;Alpha-keto-isocaproate;Alpha-keto-isocaproic acid;Alpha-ketoisocaproate;Alpha-ketoisocaproic acid;Alpha-ketoisocapronate;Alpha-ketoisocapronic acid;Alpha-oxoisocaproate;Alpha-oxoisocaproic acid;Keto-leucinePW_C000547Ketoleu1583273741887524220132323047918213212154512412410311829763-Methyl-1-hydroxybutyl-ThPPHMDB00068653-Methyl-1-hydroxybutyl-ThPP is an intermediate in valine, leucine and isoleucine degradation(KEGG ID C15974). It is the second to last step in the synthesis of 3-methylbutanoyl-CoA and is converted from 4-methyl-2-oxopentanoate via the enzyme 2-oxoisovalerate dehydrogenase [EC:1.2.4.4]. It is then converted to S-(3-methylbutanoyl)-dihydrolipoamide-E via the enzyme 2-oxoisovalerate dehydrogenase [EC:1.2.4.4].C159742372462522378483CC(C)CC(O)C1=[N+](CC2=C(N)N=C(C)N=C2)C(C)=C(CCOP(O)(=O)OP(O)(O)=O)S1C17H29N4O8P2SInChI=1S/C17H28N4O8P2S/c1-10(2)7-14(22)17-21(9-13-8-19-12(4)20-16(13)18)11(3)15(32-17)5-6-28-31(26,27)29-30(23,24)25/h8,10,14,22H,5-7,9H2,1-4H3,(H4-,18,19,20,23,24,25,26,27)/p+1OZAWOYZVNPQFFO-UHFFFAOYSA-O3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-{[hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-2-(1-hydroxy-3-methylbutyl)-4-methyl-1,3-thiazol-3-ium511.447511.118132638-3.8553-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-{[hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-2-(1-hydroxy-3-methylbutyl)-4-methyl-1,3-thiazol-3-ium1-1FDB0241213-methyl-1-hydroxybutyl-tppPW_C0029763MhThPP1587479168133121528406124086120769LipoamideHMDB0000962Lipoamide is the oxidized form of glutathione. (PMID:8957191). Lipoamide is a trivial name for 6,8-dithiooctanoic amide. It is 6,8-dithiooctanoic acid's functional form where the carboxyl group is attached to protein (or any other amine) by an amide linkage (containing -NH2) to an amino group. Lipoamide forms a thioester bond, oxidizing the disulfide bond, with acetaldehyde (pyruvate after it has been decarboxylated). It then transfers the acetaldehyde group to CoA which can then continue in the TCA cycle. (Wikipedia). Lipoamide is an intermediate in glycolysis/gluconeogenesis, citrate cycle (TCA cycle), alanine, aspartate and pyruvate metabolism, and valine, leucine and isoleucine degradation (KEGG:C00248). It is generated from dihydrolipoamide via the enzyme dihydrolipoamide dehydrogenase (EC:1.8.1.4) and then converted to S-glutaryl-dihydrolipoamide via the enzyme oxoglutarate dehydrogenase (EC:1.2.4.2).940-69-2C0024886317460LIPOAMIDE840NC(=O)CCCCC1CCSS1C8H15NOS2InChI=1S/C8H15NOS2/c9-8(10)4-2-1-3-7-5-6-11-12-7/h7H,1-6H2,(H2,9,10)FCCDDURTIIUXBY-UHFFFAOYSA-N5-(1,2-dithiolan-3-yl)pentanamide205.341205.059505487-3.311lipoamide00FDB0223401,2-dithiolane-3-pentanamide;5-(1,2-dithiolan-3-yl)-pentanamide;5-(1,2-dithiolan-3-yl)pentanamide;5-(1,2-dithiolan-3-yl)valeramide;5-(dithiolan-3-yl)valeramide;Dl-lipoamide;Dl-6-thioctic amide;Lipamide;Lipoacin;Lipoamid;Lipoicin;Lipozyme;Lypoaran;Pathoclon;Thioami;Thioctamid;Thioctamide;Thioctic acid amide;Thioctic acid amide (jan);Thiotomin;Ticolin;Vitamin n;Alpha-lipoate;Alpha-lipoic acid;Alpha-lipoic acid amide;A-lipoate amide;A-lipoic acid amide;Alpha-lipoate amide;α-lipoate amide;α-lipoic acid amide;Thioctate amidePW_C000769Lipoamd20241073317342466785367103602915560811616389164741787464222771251337828611279173132800113681199574061208034071215351241227471201233891191240931181253474791260784811268905011275342062978S-(3-Methylbutanoyl)-dihydrolipoamide-EHMDB0006867S-(3-Methylbutanoyl)-dihydrolipoamide-E is an intermediate in valine, leucine and isoleucine degradation(KEGG ID C15975 ). It is the second to last step in the synthesis of branched chain fatty acid and is converted from 3-methyl-hydroxybutyl-ThPP via the enzyme 2-oxoisovalerate dehydrogenase [EC:1.2.4.4]. It is then converted to 3-methylbutanoyl-CoA via the enzyme dihydrolipoyllysine-residue (2-methylpropanoyl)transferase[EC:2.3.1.168].C0511944056627462389466CC(C)CC(=O)SCCC(S)CCCCC(N)=OC13H25NO2S2InChI=1S/C13H25NO2S2/c1-10(2)9-13(16)18-8-7-11(17)5-3-4-6-12(14)15/h10-11,17H,3-9H2,1-2H3,(H2,14,15)KMUSXGCRMMQDBP-UHFFFAOYSA-N8-[(3-methylbutanoyl)sulfanyl]-6-sulfanyloctanamide291.473291.132670429-4.4328-[(3-methylbutanoyl)sulfanyl]-6-sulfanyloctanamide00FDB024123S-(3-methylbutanoyl)-dihydrolipoamide-e;S-(3-methylbutanoyl)dihydrolipoyllysine;[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase];S-(3-methylbutanoyl)-dihydrolipoamidePW_C002978S3MdipE15944791691331215294061240871201060Thiamine pyrophosphateHMDB0001372Thiamine pyrophosphate is the active form of thiamine, and it serves as a cofactor for several enzymes involved primarily in carbohydrate catabolism. The enzymes are important in the biosynthesis of a number of cell constituents, including neurotransmitters, and for the production of reducing equivalents used in oxidant stress defenses and in biosyntheses and for synthesis of pentoses used as nucleic acid precursors. The chemical structure of TPP is that of an aromatic methylaminopyrimidine ring, linked via a methylene bridge to a methylthiazolium ring with a pyrophosphate group attached to a hydroxyethyl side chain. In non-enzymatic model studies it has been demonstrated that the thiazolium ring can catalyse reactions which are similar to those of TPP-dependent enzymes but several orders of magnitude slower. Using infrared and NMR spectrophotometry it has been shown that the dissociation of the proton from C2 of the thiazolium ring is necessary for catalysis; the abstraction of the proton leads to the formation of a carbanion (ylid) with the potential for a nucleophilic attack on the carbonyl group of the substrate. In all TPP-dependent enzymes the abstraction of the proton from the C2 atom is the first step in catalysis, which is followed by a nucleophilic attack of this carbanion on the substrate. Subsequent cleavage of a C-C bond releases the first product with formation of a second carbanion (2-greek small letter alpha-carbanion or enamine). The formation of this 2-greek small letter alpha-carbanion is the second feature of TPP catalysis common to all TPP-dependent enzymes. Depending on the enzyme and the substrate(s), the reaction intermediates and products differ. Methyl-branched fatty acids, as phytanic acid, undergo peroxisomal beta-oxidation in which they are shortened by 1 carbon atom. This process includes four steps: activation, 2-hydroxylation, thiamine pyrophosphate dependent cleavage and aldehyde dehydrogenation. In the third step, 2-hydroxy-3-methylacyl-CoA is cleaved in the peroxisomal matrix by 2-hydroxyphytanoyl-CoA lyase (2-HPCL), which uses thiamine pyrophosphate (TPP) as cofactor. The thiamine pyrophosphate dependence of the third step is unique in peroxisomal mammalian enzymology. Human pathology due to a deficient alpha-oxidation is mostly linked to mutations in the gene coding for the second enzyme of the sequence, phytanoyl-CoA hydroxylase (EC 1.14.11.18). (PMID: 12694175, 11899071, 9924800).154-87-0C00068113295322-(alpha-lactyl)-thpp1100CC1=C(CCOP(O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1NC12H19N4O7P2SInChI=1S/C12H18N4O7P2S/c1-8-11(3-4-22-25(20,21)23-24(17,18)19)26-7-16(8)6-10-5-14-9(2)15-12(10)13/h5,7H,3-4,6H2,1-2H3,(H4-,13,14,15,17,18,19,20,21)/p+1AYEKOFBPNLCAJY-UHFFFAOYSA-O3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-{[hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-4-methyl-1,3-thiazol-3-ium425.314425.044967696-3.484thiamin pyrophosphate1-1FDB022584Tpp;Thpp;Thaimine pyrophosphate;Thiamin diphosphate;Thiamin pyrophosphate;Thiamin-ppi;Thiamine diphosphate;Thiamine pyrophosphate;Thiamine-ppi;Thiamine-pyrophosphate;Thiamin diphosphoric acid;Thiamine(1+) diphosphoric acid;Thiamin pyrophosphoric acid;Thiamine diphosphoric acidPW_C001060ThiamPP205410753119781271517362536610360281556080161638816473178746322212806225771241337828511278423334790181117917513280010368119956406120802407120902122120982408121537124122746120123388119123473135123547374124095118125346479125922482126094481126802299126889501127381502127549206128400388870Isovaleryl-CoAHMDB0001113Isovaleryl-CoA is an intermediate metabolite in the catabolic pathway of leucine. The accumulation of derivatives of isovaleryl-CoA occurs in patients affected with isovaleric acidemia (IVA, OMIM 243500) an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD, EC 1.3.99.10, a flavoenzyme that catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA). IVA was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. Both intra- and interfamilial variability have been recognized. Initially, two phenotypes with either an acute neonatal or a chronic intermittent presentation were described. More recently, a third group of individuals with mild biochemical abnormalities who can be asymptomatic have been identified through newborn screening of blood spots by tandem mass spectrometry. The majority of patients with IVA today are diagnosed pre-symptomatically through newborn screening by use of MS/MS which reveals elevations of the marker metabolite C5 acylcarnitine in dried blood spots. C5 acylcarnitine represents a mixture of isomers (isovalerylcarnitine, 2-methylbutyrylcarnitine, and pivaloylcarnitine). (PMID: 16602101, Am J Med Genet C Semin Med Genet. 2006 May 15;142(2):95-103.).6244-91-3C0293943985515487ISOVALERYL-COA388897CC(C)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC26H44N7O17P3SInChI=1S/C26H44N7O17P3S/c1-14(2)9-17(35)54-8-7-28-16(34)5-6-29-24(38)21(37)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-20(49-51(39,40)41)19(36)25(48-15)33-13-32-18-22(27)30-12-31-23(18)33/h12-15,19-21,25,36-37H,5-11H2,1-4H3,(H,28,34)(H,29,38)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41)/t15-,19-,20-,21?,25-/m1/s1UYVZIWWBJMYRCD-TVCSPYKZSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid851.651851.172723243-2.179[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB0224303-methylbutanoyl-coa;3-methylbutanoyl-coenzyme a;3-methylbutyryl-coa;3-methylbutyryl-coenzyme a;Isovaleryl coa;Isovaleryl coenzyme a;Isovaleryl-coenzyme a;S-(3-methylbutanoate;S-(3-methylbutanoate) coenzyme a;S-(3-methylbutanoic acid;S-isovalerate coa;S-isovalerate coenzyme aPW_C000870IsovCoA159544361379185133121550406124108120782DihydrolipoamideHMDB0000985Dihydrolipoamide is an intermediate in glycolysis/gluconeogenesis, citrate cycle (TCA cycle), alanine, aspartate and pyruvate metabolism, and valine, leucine and isoleucine degradation (KEGG ID C00579). It is converted to lipoamide via the enzyme dihydrolipoamide dehydrogenase [EC:1.8.1.4]. Dihydrolipoamide is also a substrate of enzyme Acyltransferases [EC 2.3.1.-]. (KEGG).3884-47-7C0057966317694DIHYDROLIPOAMIDE643OC(=N)CCCCC(S)CCSC8H17NOS2InChI=1S/C8H17NOS2/c9-8(10)4-2-1-3-7(12)5-6-11/h7,11-12H,1-6H2,(H2,9,10)VLYUGYAKYZETRF-UHFFFAOYSA-N6,8-disulfanyloctanimidic acid207.357207.075155551-3.324dihydrothioctamide00FDB0223526,8-bis-sulfanyloctanamide;6,8-dimercapto-octanamide;6,8-dimercaptooctanamide;6,8-disulfanyloctanamide;Dihydrolipoamide;Dihydrothioctamide;6,8-bis(sulphanyl)octanamidePW_C000782DHLipoa2124107837877411279170133121451407121532406124009119124090120126077481127533206721NADHMDB0000902NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).53-84-9C00003589315846NAD5682NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP([O-])(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1OC21H27N7O14P2InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-N1-[(2R,3R,4S,5R)-5-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy)(hydroxy)phosphoryl]oxy}methyl)-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium663.4251663.109121631-2.5281-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium0-1FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571851144NADHHMDB0001487NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).58-68-4C0000443915316908NADH388299DB00157NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O14P2InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BOPGDPNILDQYTO-NNYOXOHSSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]({[(2R,3S,4R,5R)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy})phosphinic acid665.441665.124771695-2.358NADH0-2FDB0226491,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotidePW_C001144NADH1434153349086481011152127551469542230492781172836293109948061848121848212849046495931516995524010353321115358112546612354791255593135569810057371085829141591514759451516027155607916163871647217867711176893160701118870991637172205719520674622228244226836022590862241180919811821216123202491300329813015300132552234240332242618315771071327712313377208134773713317765133677668334777003327770713077917113779863478000936880691119938221241105493881128549411583811811995540612017240712037812212098640812116242512124412612169342912181838312261638412274512012312744712313813612355137412373446012381444312424246412437139812518912112534547912553148112576229712580829912592648212651649512676748012688850112738550212809039012836239112842939514075918540034Hydrogen IonHMDB0059597Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])C000801038153781010[H+]HInChI=1S/p+1GPRLSGONYQIRFK-UHFFFAOYSA-Nhydron1.00791.0078250320hydron10H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+21546708753157883184831116214632614645422314927801742502242544245471045761846947052411035327111535311256261085639107569910057201055742117596314760371556070157609316161301596232166648317866011526692101684318869101877100163716820571912067453219745422074722227525213753221075582127572160759017081952258218151824322684131628420224913919591552491191516412015281121812851224628612266287125212271325722313325294153303084232931542354318424013224240531242454320769122937713613377210134773723317780411477955132779903277799134778379345799291308001936880387310803883048072211993823124948233831105503881128559411328039011553739811553911811585633611620510911997340612019340712054912212059340912117042412117142512256941812261538412268712512275812012318313512321813712374245912374346012514145412518812112527313612535947912555048112573048312573629712580929912651749512671748912676648012682330012690250112721320812830850612836139112843039514069288214069388314069916714070716814071514140742788140743597140760185964FADHMDB0001248FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).146-14-5C0001664397516238FAD559059DB03147CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD999114518681923216425317628288251884021188141489421612291622492133582536223723264602364688314741134758104881652681035285102533511154961265511127561311860301556054156608216161161626390164751786499179666610770391637175205732121374652227487223907622411818216118872151189921112296225123282491244315112519227125952261271029112720292130293011304130243623318770802937712613377152134775011137750711277518115775413347761513277726337780543297837534578930331792223367927235880012368800343698071411911995840611999938412005140812010740712043240512045312212049012412127842912129841812141738212148938312274812012277612112280237412282344312306637612308713512316644812384946412386845412397639912404739812534847912537848012542948212547448112569729712597948912610729912627748412689150112692039112696850212698720712701120612731020912743250612760238812784038914079018514079918611503-Methylcrotonyl-CoAHMDB00014933-Methylcrotonyl-CoA, also known as 3-methylbut-2-enoyl-CoA or dimethylacryloyl-CoA, belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. 3-Methylcrotonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). 3-Methylcrotonyl-CoA has been primarily detected in urine. Within the cell, 3-methylcrotonyl-CoA is primarily located in the mitochondria and cytoplasm. 3-Methylcrotonyl-CoA exists in all living organisms, ranging from bacteria to humans. In humans, 3-methylcrotonyl-CoA is involved in the valine, leucine and isoleucine degradation pathway. 3-Methylcrotonyl-CoA is also involved in several metabolic disorders, some of which include Beta-ketothiolase deficiency, the propionic acidemia pathway, the 3-hydroxyisobutyric aciduria pathway, and 2-methyl-3-hydroxybutryl CoA dehydrogenase deficiency. 3-Methylcrotonyl-CoA is an essential metabolite for leucine metabolism, a substrate of 3-methylcrotonyl-CoA carboxylase (EC 6.4.1.4), a biotin-dependent mitochondrial enzyme in the catabolism of leucine. (OMIM 609010).793193-48-3C03069439869154863-METHYL-CROTONYL-COA388909CC(C)=CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC26H42N7O17P3SInChI=1S/C26H42N7O17P3S/c1-14(2)9-17(35)54-8-7-28-16(34)5-6-29-24(38)21(37)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-20(49-51(39,40)41)19(36)25(48-15)33-13-32-18-22(27)30-12-31-23(18)33/h9,12-13,15,19-21,25,36-37H,5-8,10-11H2,1-4H3,(H,28,34)(H,29,38)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41)/t15-,19-,20-,21?,25-/m1/s1BXIPALATIYNHJN-TVCSPYKZSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-methylbut-2-enoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid849.635849.157073179-2.199[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-methylbut-2-enoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB0226523-methylbut-2-enoyl-coa;3-methylbut-2-enoyl-coenzyme a;3-methylcrotonoyl-coa;3-methylcrotonoyl-coenzyme a;3-methylcrotonyl-coa;3-methylcrotonyl-coenzyme a;Dimethylacryloyl-coa;Dimethylacryloyl-coenzyme a;B-methylcrotonyl-coa (hmdb01493;B-methylcrotonyl-coenzyme a (hmdb01493;Beta-methylcrotonoyl-coa;Beta-methylcrotonoyl-coenzyme a;Beta-methylcrotonyl-coa (hmdb01493;Beta-methylcrotonyl-coenzyme a (hmdb01493PW_C0011503McroCA1600443623791871331215534061241111208323-Methylglutaconyl-CoAHMDB00010573-Methylglutaconyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. Thus, 3-methylglutaconyl-CoA is considered to be a fatty ester lipid molecule. 3-Methylglutaconyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). 3-Methylglutaconyl-CoA has been primarily detected in urine. Within the cell, 3-methylglutaconyl-CoA is primarily located in the mitochondria and cytoplasm. In humans, 3-methylglutaconyl-CoA is involved in the valine, leucine and isoleucine degradation pathway. 3-Methylglutaconyl-CoA is also involved in several metabolic disorders, some of which include 2-methyl-3-hydroxybutryl CoA dehydrogenase deficiency, 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, the methylmalonic aciduria pathway, and the 3-methylglutaconic aciduria type III pathway. 3-Methylglutaconyl-CoA is a substrate for Methylglutaconyl-CoA hydratase (mitochondrial), Methylcrotonoyl-CoA carboxylase beta chain (mitochondrial) and Methylcrotonoyl-CoA carboxylase alpha chain (mitochondrial).6247-73-0C03231546221415488TRANS-3-METHYL-GLUTACONYL-COA11471767C\C(CC(O)=O)=C/C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C27H42N7O19P3SInChI=1S/C27H42N7O19P3S/c1-14(8-17(36)37)9-18(38)57-7-6-29-16(35)4-5-30-25(41)22(40)27(2,3)11-50-56(47,48)53-55(45,46)49-10-15-21(52-54(42,43)44)20(39)26(51-15)34-13-33-19-23(28)31-12-32-24(19)34/h9,12-13,15,20-22,26,39-40H,4-8,10-11H2,1-3H3,(H,29,35)(H,30,41)(H,36,37)(H,45,46)(H,47,48)(H2,28,31,32)(H2,42,43,44)/b14-9+GXKSHRDAHFLWPN-NTEUORMPSA-N(3E)-5-({2-[(3-{[4-({[({[5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)-1,2-dihydroxy-3,3-dimethylbutylidene]amino}-1-hydroxypropylidene)amino]ethyl}sulfanyl)-3-methyl-5-oxopent-3-enoic acid893.644893.146902423-2.2410(3E)-5-[(2-{[3-({4-[({[5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]-1,2-dihydroxy-3,3-dimethylbutylidene}amino)-1-hydroxypropylidene]amino}ethyl)sulfanyl]-3-methyl-5-oxopent-3-enoic acid0-5FDB0223963-methylglutaconyl-coa;3-methylglutaconyl-coenzyme a;Trans-3-methylglutaconyl-coa;Trans-3-methylglutaconyl-coenzyme aPW_C0008323MCoA160447918913312155840612411612010633-Hydroxy-3-methylglutaryl-CoAHMDB00013753-hydroxy-3-methylglutaryl CoA (HMG-CoA) is formed when Acetyl-CoA condenses with acetoacetyl-CoA in a reaction that is catalyzed by the enzyme HMG-CoA synthase in the mevalonate pathway or mevalonate-dependent (MAD) route, an important cellular metabolic pathway present in virtually all organisms. HMG-CoA reductase (EC 1.1.1.34) inhibitors, more commonly known as statins, are cholesterol-lowering drugs that have been widely used for many years to reduce the incidence of adverse cardiovascular events. HMG-CoA reductase catalyzes the rate-limiting step in the mevalonate pathway and these agents lower cholesterol by inhibiting its synthesis in the liver and in peripheral tissues. Androgen also stimulates lipogenesis in human prostate cancer cells directly by increasing transcription of the fatty acid synthase and HMG-CoA-reductase genes. (PMID: 14689582).1553-55-5C00356439218154673-HYDROXY-3-METHYL-GLUTARYL-COA388357C[C@](O)(CC(O)=O)CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC27H44N7O20P3SInChI=1S/C27H44N7O20P3S/c1-26(2,21(40)24(41)30-5-4-15(35)29-6-7-58-17(38)9-27(3,42)8-16(36)37)11-51-57(48,49)54-56(46,47)50-10-14-20(53-55(43,44)45)19(39)25(52-14)34-13-33-18-22(28)31-12-32-23(18)34/h12-14,19-21,25,39-40,42H,4-11H2,1-3H3,(H,29,35)(H,30,41)(H,36,37)(H,46,47)(H,48,49)(H2,28,31,32)(H2,43,44,45)/t14-,19-,20-,21+,25-,27+/m1/s1CABVTRNMFUVUDM-VRHQGPGLSA-N(3S)-5-[(2-{3-[(2R)-3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-3-hydroxy-3-methyl-5-oxopentanoic acid911.659911.157467109-2.3511HMG-CoA0-5FDB022587(s)-3-hydroxy-3-methylglutaryl-coa;(s)-3-hydroxy-3-methylglutaryl-coenzyme a;3-hydroxy-3-methyl-glutaryl-coa;3-hydroxy-3-methyl-glutaryl-coenzyme a;3-hydroxy-3-methylglutaryl-coa;3-hydroxy-3-methylglutaryl-coenzyme a;Hmg-coa;Hmg-coenzyme a;Hydroxymethylglutaroyl coenzyme a;Hydroxymethylglutaryl-coa;Hydroxymethylglutaryl-coenzyme a;S-(hydrogen 3-hydroxy-3-methylglutaryl)coenzyme a;S-(hydrogen 3-hydroxy-3-methylpentanedioate;S-(hydrogen 3-hydroxy-3-methylpentanedioate) coenzyme a;S-(hydrogen 3-hydroxy-3-methylpentanedioic acidPW_C001063HMG-CoA5954794210513152087296198735816382782101524015115241222776931337822511278916111120509406120765407121467122123115120123361119124025135940Acetyl-CoAHMDB0001206The main function of coenzyme A is to carry acyl groups (such as the acetyl group) or thioesters. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA, which is a vital component in cholesterol and ketone synthesis. (wikipedia). acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.72-89-9C0002444449315351ACETYL-COA392413CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC23H38N7O17P3SInChI=1S/C23H38N7O17P3S/c1-12(31)51-7-6-25-14(32)4-5-26-21(35)18(34)23(2,3)9-44-50(41,42)47-49(39,40)43-8-13-17(46-48(36,37)38)16(33)22(45-13)30-11-29-15-19(24)27-10-28-20(15)30/h10-11,13,16-18,22,33-34H,4-9H2,1-3H3,(H,25,32)(H,26,35)(H,39,40)(H,41,42)(H2,24,27,28)(H2,36,37,38)/t13-,16-,17-,18+,22-/m1/s1ZSLZBFCDCINBPY-ZSJPKINUSA-N{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid809.571809.125773051-2.279acetyl-CoA0-4FDB022491Ac-coa;Ac-coenzyme a;Ac-s-coa;Ac-s-coenzyme a;Acetyl coenzyme-a;Acetyl-coa;Acetyl-coenzyme a;Acetyl-s-coa;Acetyl-s-coenzyme a;Acetylcoenzyme-a;S-acetate coa;S-acetate coenzyme a;S-acetyl coenzyme a;Accoa;Acetyl coenzyme a;S-acetyl-coa;S-acetyl-coenzyme a;Acetylcoenzyme aPW_C000940Ac-CoA213438588423241622446528961733401148401452781035476124573310860251556077161638616470178692316071061637291198746022282451518277210125822261301229942615315771211337729111177562112777061327799411578355134784333348000736880634119806633769012417011995340612014540512030412212063240712241740812262638412274312012295913512313711812498637412520012112534347912550747812563329712656448212657248112677848012688650112704420912739420512766538812813750212814520612837439114076218542Acetoacetic acidHMDB0000060Acetoacetic acid (AcAc) is a weak organic acid that can be produced in the human liver under certain conditions of poor metabolism leading to excessive fatty acid breakdown (diabetes mellitus leading to diabetic ketoacidosis). It is then partially converted into acetone by decarboxylation and excreted either in urine or through respiration. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing rats. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate and beta-hydroxybutyrate are preferred over glucose as substrates for synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first two weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies are utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmityl phosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life (PMID: 3884391). The acid is also present in the metabolism of those undergoing starvation or prolonged physical exertion as part of gluconeogenesis. When ketone bodies are measured by way of urine concentration, acetoacetic acid, along with beta-hydroxybutyric acid or acetone, is what is detected.541-50-4C0016496153443-KETOBUTYRATE94DB01762CC(=O)CC(O)=OC4H6O3InChI=1S/C4H6O3/c1-3(5)2-4(6)7/h2H2,1H3,(H,6,7)WDJHALXBUFZDSR-UHFFFAOYSA-N3-oxobutanoic acid102.0886102.0316940580.371acetoacetic acid0-1FDB0218013-ketobutyrate;3-ketobutyric acid;3-oxo-butanoate;3-oxo-butanoic acid;3-oxobutyrate;3-oxobutyric acid;Acetoacetate;Diacetate;Diacetic acid;3-oxobutanoic acid;Beta-ketobutyric acid;3-oxobutanoate;B-ketobutyrate;B-ketobutyric acid;Beta-ketobutyrate;β-ketobutyrate;β-ketobutyric acidPW_C000042LIN597410533130282003269291607360163906615115242222776941337783513278227112784831111205114061207674071210191221215981241231171201233631191235841351241561181266092991275643881142Acetoacetyl-CoAHMDB0001484Acetoacetyl-CoA is an intermediate in the metabolism of Butanoate. It is a substrate for Succinyl-CoA:3-ketoacid-coenzyme A transferase 1 (mitochondrial), Hydroxymethylglutaryl-CoA synthase (mitochondrial), Short chain 3-hydroxyacyl-CoA dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), Hydroxymethylglutaryl-CoA synthase (cytoplasmic), Peroxisomal bifunctional enzyme, Acetyl-CoA acetyltransferase (cytosolic), Acetyl-CoA acetyltransferase (mitochondrial), 3-hydroxyacyl-CoA dehydrogenase type II, Succinyl-CoA:3-ketoacid-coenzyme A transferase 2 (mitochondrial), 3-ketoacyl-CoA thiolase (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal) and Trifunctional enzyme alpha subunit (mitochondrial).1420-36-6C0033243921415345ACETOACETYL-COA388353CC(=O)CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC25H40N7O18P3SInChI=1S/C25H40N7O18P3S/c1-13(33)8-16(35)54-7-6-27-15(34)4-5-28-23(38)20(37)25(2,3)10-47-53(44,45)50-52(42,43)46-9-14-19(49-51(39,40)41)18(36)24(48-14)32-12-31-17-21(26)29-11-30-22(17)32/h11-12,14,18-20,24,36-37H,4-10H2,1-3H3,(H,27,34)(H,28,38)(H,42,43)(H,44,45)(H2,26,29,30)(H2,39,40,41)/t14-,18-,19-,20+,24-/m1/s1OJFDKHTZOUZBOS-CITAKDKDSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-oxobutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid851.607851.136337737-2.169acetoacetyl-coa0-4FDB0226483-acetoacetyl-coa;3-acetoacetyl-coenzyme a;3-oxobutyryl-coa;3-oxobutyryl-coenzyme a;Acetoacetyl coa;Acetoacetyl coenzyme a;Acetoacetyl-coa;Acetoacetyl-coenzyme a;S-acetoacetylcoenzyme aPW_C001142ActaCoA592479281049352791037002161729219873571637598160824222683062101523915177258133782241127891411190126170120283406120763407121465122122948120123359119124023135125622479126085481127149501127541206140796185704L-ValineHMDB0000883Valine (abbreviated as Val or V) is an -amino acid with the chemical formula HO2CCH(NH2)CH(CH3)2. It is named after the plant valerian. L-Valine is one of 20 proteinogenic amino acids. Its codons are GUU, GUC, GUA, and GUG. This essential amino acid is classified as nonpolar. Along with leucine and isoleucine, valine is a branched-chain amino acid. Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)tyrosine, tryptophan and phenylalanine, as well as methionineare increased in these conditions. Valine in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. (http://www.dcnutrition.com). In sickle-cell disease, valine substitutes for the hydrophilic amino acid glutamic acid in hemoglobin. Because valine is hydrophobic, the hemoglobin does not fold correctly. Valine is an essential amino acid, hence it must be ingested, usually as a component of proteins.72-18-4C00183628716414VAL6050DB00161CC(C)[C@H](N)C(O)=OC5H11NO2InChI=1S/C5H11NO2/c1-3(2)4(6)5(7)8/h3-4H,6H2,1-2H3,(H,7,8)/t4-/m0/s1KZSNJWFQEVHDMF-BYPYZUCNSA-N(2S)-2-amino-3-methylbutanoic acid117.1463117.0789786010.262L-valine00FDB000465(2s)-2-amino-3-methylbutanoate;(2s)-2-amino-3-methylbutanoic acid;(s)-2-amino-3-methylbutanoate;(s)-2-amino-3-methylbutanoic acid;(s)-2-amino-3-methylbutyrate;(s)-2-amino-3-methylbutyric acid;(s)-2-amino-3-methyl-butanoate;(s)-2-amino-3-methyl-butanoic acid;(s)-valine;(s)-a-amino-b-methylbutyrate;(s)-a-amino-b-methylbutyric acid;(s)-alpha-amino-beta-methylbutyrate;(s)-alpha-amino-beta-methylbutyric acid;2-amino-3-methylbutanoate;2-amino-3-methylbutanoic acid;2-amino-3-methylbutyrate;2-amino-3-methylbutyric acid;L-(+)-a-aminoisovalerate;L-(+)-a-aminoisovaleric acid;L-(+)-alpha-aminoisovalerate;L-(+)-alpha-aminoisovaleric acid;L-valine;L-a-amino-b-methylbutyrate;L-a-amino-b-methylbutyric acid;L-alpha-amino-beta-methylbutyrate;L-alpha-amino-beta-methylbutyric acid;Valine;L-valin;V;ValPW_C000704Val1651823134565310756541087144187906922490701519071225422583104254131542560318786251337917811112154012212225440612409813512480712012641647912798250112α-Ketoisovaleric acidHMDB0000019alpha-Ketoisovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. alpha-Ketoisovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of alpha-ketoisovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465). alpha-Ketoisovaleric acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development.759-05-7C0014149165302-KETO-ISOVALERATE48DB04074CC(C)C(=O)C(O)=OC5H8O3InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)QHKABHOOEWYVLI-UHFFFAOYSA-N3-methyl-2-oxobutanoic acid116.1152116.047344122-0.591α-ketoisovalerate0-1FDB0122502-keto-3-methylbutyrate;2-keto-3-methylbutyric acid;2-ketoisovalerate;2-ketoisovaleric acid;2-ketoisvaleric acid;2-oxo-3-methyl-butyrate;2-oxo-3-methylbutanoate;2-oxo-3-methylbutanoic acid;2-oxo-3-methylbutyrate;2-oxo-3-methylbutyric acid;2-oxoisovalerate;2-oxoisovaleric acid;3-methyl-2-oxo-butanoate;3-methyl-2-oxo-butanoic acid;3-methyl-2-oxo-butyrate;3-methyl-2-oxo-butyric acid;3-methyl-2-oxobutanoate;3-methyl-2-oxobutanoic acid;3-methyl-2-oxobutinoate;3-methyl-2-oxobutinoic acid;3-methyl-2-oxobutyrate;3-methyl-2-oxobutyric acid;Dimethylpyruvate;Dimethylpyruvic acid;Isopropylglyoxylate;Isopropylglyoxylic acid;Ketovaline;A-keto-isovalerate;A-keto-isovaleric acid;A-keto-b-methylbutyrate;A-keto-b-methylbutyric acid;A-ketoisovalerate;A-ketoisovaleric acid;A-oxo-b-methylbutyrate;A-oxo-b-methylbutyric acid;A-oxoisovalerate;A-oxoisovaleric acid;Alpha-keto-isovalerate;Alpha-keto-isovaleric acid;Alpha-keto-beta-methylbutyrate;Alpha-keto-beta-methylbutyric acid;Alpha-ketoisovalerate;Alpha-ketoisovaleric acid;Alpha-oxo-beta-methylbutyrate;Alpha-oxo-beta-methylbutyric acid;Alpha-oxoisovalerate;Alpha-oxoisovaleric acid;2-ketovaline;Alpha-ketovaline;Alpha-oxo-beta-methylbutyricacid;α-keto-isovalerate;α-keto-isovaleric acid;A-ketovaline;α-ketovaline;A-oxo-b-methylbutyricacid;α-oxo-β-methylbutyricacid;α-oxoisovalerate;α-oxoisovaleric acidPW_C000012Ketoval165285784108706418875811639068220426383157917911112154112212409913529772-Methyl-1-hydroxypropyl-ThPP HMDB00068662-Methyl-1-hydroxypropyl-ThPP is an intermediate in valine, leucine and isoleucine degradation(KEGG ID C15976). It is the second to last step in the synthesis of isobutyryl-CoA and is converted from 3-methyl-2-oxobutanoate via the enzyme 2-oxoisovalerate dehydrogenase [EC:1.2.4.4]. It is then converted to S-(2-methylpropanoyl)-dihydrolipoamide-E via the enzyme 2-oxoisovalerate dehydrogenase [EC:1.2.4.4]].C15976237246264852226333171CC(C)C(O)C1=[N+](CC2=C(N)N=C(C)N=C2)C(C)=C(CCOP(O)(=O)OP(O)(O)=O)S1C16H27N4O8P2SInChI=1S/C16H26N4O8P2S/c1-9(2)14(21)16-20(8-12-7-18-11(4)19-15(12)17)10(3)13(31-16)5-6-27-30(25,26)28-29(22,23)24/h7,9,14,21H,5-6,8H2,1-4H3,(H4-,17,18,19,22,23,24,25,26)/p+1SSYCSHKTIOHFEZ-UHFFFAOYSA-O3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-{[hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-2-(1-hydroxy-2-methylpropyl)-4-methyl-1,3-thiazol-3-ium497.42497.102482574-3.7953-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-{[hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-2-(1-hydroxy-2-methylpropyl)-4-methyl-1,3-thiazol-3-ium1-1FDB0241222-methyl-1-hydroxypropyl-tpp;1-hydroxy-2-methylpropyl-thiamine diphosphate;1-hydroxy-2-methylpropyl-thiamine pyrophosphate;2-methyl-1-hydroxypropyl-thiamine diphosphate;2-methyl-1-hydroxypropyl-thiamine pyrophosphate;3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-diphosphoethyl)-2-(1-hydroxy-2-methylpropyl)-4-methyl-1,3-thiazol-3-ium;1-hydroxy-2-methylpropyl-thiamine diphosphoric acid;2-methyl-1-hydroxypropylthiamine diphosphoric acid;1-hydroxy-2-methylpropyl-thiamine pyrophosphoric acid;2-methyl-1-hydroxypropyl-thiamine diphosphoric acid;2-methyl-1-hydroxypropyl-thiamine pyrophosphoric acidPW_C0029772M1ThPP16564791711331215334061240911202979S-(2-Methylpropionyl)-dihydrolipoamide-EHMDB0006868S-(2-Methylpropionyl)-dihydrolipoamide-E is an intermediate in valine, leucine and isoleucine degradation(KEGG ID C15977). It is the second to last step in the synthesis of branched chain fatty acid and is converted from 2-methyl-1-hydroxypropyl-ThPP via the enzyme 2-oxoisovalerate dehydrogenase [EC:1.2.4.4]. It is then converted to isobutyryl-CoA via the enzyme dihydrolipoyllysine-residue (2-methylpropanoyl)transferase [EC:2.3.1.168].C04424119538351757710128135CC(C)C(=O)SCCC(S)CCCCC(N)=OC12H23NO2S2InChI=1S/C12H23NO2S2/c1-9(2)12(15)17-8-7-10(16)5-3-4-6-11(13)14/h9-10,16H,3-8H2,1-2H3,(H2,13,14)UEFURMXXHJCLJP-UHFFFAOYSA-N8-[(2-methylpropanoyl)sulfanyl]-6-sulfanyloctanamide277.447277.117020365-4.2528-[(2-methylpropanoyl)sulfanyl]-6-sulfanyloctanamide00FDB024124S-(2-methylpropanoyl)-dihydrolipoamide-e;S-(2-methylpropionyl)-dihydrolipoamide-e;[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]s-(2-methylpropanoyl)dihydrolipoyllysine;S-(2-methylpropanoyl)-dihydrolipoamide;S-(2-methylpropionyl)-dihydrolipoamidePW_C002979S2MDE1657479172133121534406124092120960Isobutyryl-CoAHMDB0001243Isobutyryl-CoA, also known as isobutanoyl-coa, belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. Thus, isobutyryl-CoA is considered to be a fatty ester lipid molecule. Isobutyryl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Isobutyryl-CoA has been primarily detected in urine. Within the cell, isobutyryl-CoA is primarily located in the mitochondria and cytoplasm. Isobutyryl-CoA exists in all living organisms, ranging from bacteria to humans. In humans, isobutyryl-CoA is involved in the phytanic Acid peroxisomal oxidation pathway and the valine, leucine and isoleucine degradation pathway. Isobutyryl-CoA is also involved in several metabolic disorders, some of which include methylmalonate semialdehyde dehydrogenase deficiency, the maple syrup urine disease pathway, the 3-methylglutaconic aciduria type I pathway, and the isovaleric acidemia pathway. Isobutyryl-CoA is a substrate for Acyl-CoA dehydrogenase (short-chain specific, mitochondrial), Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial) and Acyl-CoA dehydrogenase (long-chain specific, mitochondrial).15621-60-0C00630303693115479ISOBUTYRYL-COA2300823CC(C)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC25H42N7O17P3SInChI=1S/C25H42N7O17P3S/c1-13(2)24(37)53-8-7-27-15(33)5-6-28-22(36)19(35)25(3,4)10-46-52(43,44)49-51(41,42)45-9-14-18(48-50(38,39)40)17(34)23(47-14)32-12-31-16-20(26)29-11-30-21(16)32/h11-14,17-19,23,34-35H,5-10H2,1-4H3,(H,27,33)(H,28,36)(H,41,42)(H,43,44)(H2,26,29,30)(H2,38,39,40)/t14-,17-,18-,19+,23-/m1/s1AEWHYWSPVRZHCT-NDZSKPAWSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-{[2-({2-[(2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid837.624837.157073179-2.349isobutyryl- coa0-4FDB0225082-methylpropanoyl-coa;2-methylpropanoyl-coenzyme a;2-methylpropionyl-coa;2-methylpropionyl-coenzyme a;Isobutyryl- coa;Isobutyryl- coenzyme a;Isobutyryl-coa;S-(2-methylpropanoate;S-(2-methylpropanoate)coa;S-(2-methylpropanoate)coenzyme a;S-(2-methylpropanoic acid;S-(2-methylpropanoyl)-coa;S-(2-methylpropanoyl)-coenzyme a;Alpha-methylpropionyl-coa;Alpha-methylpropionyl-coenzyme a;Isobutanoyl-coaPW_C000960IsoBCoA12788166847855711179190133120996122121562406123561135124120120125936297127396205800Methacrylyl-CoAHMDB0001011Methacrylyl-CoA is a metabolite in the valine, leucine and isoleucine degradation pathway and highly reacts with free thiol compounds (PMID 14684172; KEGG). Cirrhosis results in a significant decrease in 3--hydroxyisobutyryl-CoA hydrolase activity, a key enzyme in the valine catabolic pathway that plays an important role in the catabolism of a potentially toxic compound, methacrylyl-CoA, formed as an intermediate in the catabolism of valine and isobutyrate. (PMID: 8938168).6008-91-9C0346044002127754METHACRYLYL-COA389035CC(=C)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC25H40N7O17P3SInChI=1S/C25H40N7O17P3S/c1-13(2)24(37)53-8-7-27-15(33)5-6-28-22(36)19(35)25(3,4)10-46-52(43,44)49-51(41,42)45-9-14-18(48-50(38,39)40)17(34)23(47-14)32-12-31-16-20(26)29-11-30-21(16)32/h11-12,14,17-19,23,34-35H,1,5-10H2,2-4H3,(H,27,33)(H,28,36)(H,41,42)(H,43,44)(H2,26,29,30)(H2,38,39,40)/t14-,17-,18-,19?,23-/m1/s1NPALUEYCDZWBOV-NNYIDDMCSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(2-methylprop-2-enoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid835.608835.141423115-2.169[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(2-methylprop-2-enoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB0223692-methylprop-2-enoyl-coa;2-methylprop-2-enoyl-coenzyme a;Methacrylyl coa;Methacrylyl coenzyme a;Methacrylyl-coenzyme a;Methylacrylyl-coa;Methylacrylyl-coenzyme a;S-(2-methyl-2-propenoate;S-(2-methyl-2-propenoate) coa;S-(2-methyl-2-propenoate) coenzyme a;S-(2-methyl-2-propenoic acidPW_C000800McryCoA16704907822479192133121564406124122120829(S)-3-Hydroxyisobutyryl-CoAHMDB0001052(S)-3-Hydroxyisobutyryl-CoA is s metabolite of 3-hydroxyisobutyryl-CoA hydrolase (EC 3.1.2.4 ) during beta-alanine metabolism (KEGG 00410), propanoate metabolism (KEGG 00640), and valine, leucine and isoleucine degradation (KEGG 00280). Deficiencies of this enzyme in valine degradation can result in hypotonia, poor feeding, motor delay, and subsequent neurological regression in infancy, episodes of ketoacidosis and Leigh-like changes in the basal ganglia on a magnetic resonance imaging scan (PMID 17160907).319440-43-2C0600088282593-HYDROXY-ISOBUTYRYL-COA86CC(CO)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C25H42N7O18P3SInChI=1S/C25H42N7O18P3S/c1-13(8-33)24(38)54-7-6-27-15(34)4-5-28-22(37)19(36)25(2,3)10-47-53(44,45)50-52(42,43)46-9-14-18(49-51(39,40)41)17(35)23(48-14)32-12-31-16-20(26)29-11-30-21(16)32/h11-14,17-19,23,33,35-36H,4-10H2,1-3H3,(H,27,34)(H,28,37)(H,42,43)(H,44,45)(H2,26,29,30)(H2,39,40,41)WWEOGFZEFHPUAM-UHFFFAOYSA-N{[5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid853.623853.151987801-2.3110[5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB022393(s)-3-hydroxyisobutyryl-coa;(s)-3-hydroxyisobutyryl-coenzyme a;3-hydroxy-2-methylpropanoyl-coa;3-hydroxy-2-methylpropanoyl-coenzyme a;3-hydroxy-2-methylpropionyl-coa;3-hydroxy-2-methylpropionyl-coenzyme a;3-hydroxy-isobutyryl-coa;3-hydroxy-isobutyryl-coenzyme a;3-hydroxyisobutyryl-coa;3-hydroxyisobutyryl-coenzyme aPW_C0008293HIsoCA1672490801517919313312156740612412412016(S)-3-Hydroxyisobutyric acidHMDB0000023(S)-3-Hydroxyisobutyric (3-HIBA) acid is an organic acid. 3-HIBA is an intermediate in L-valine metabolism. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM 236795) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM 603178). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. 3-hydroxyisobutyric aciduria is caused by a 3-hydroxyisobutyryl-CoA dehydrogenase deficiency (PMID: 18329219). The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears and micrognathia (PMID: 113770040, 10686279). Lactic acidemia is also found in the affected patients, indicating that mitochondrial dysfunction is involved. 3-hydroxyisobutyrate appears to specifically inhibit the function of the respiratory chain complex I-III and mitochondrial creatine kinase (PMID: 18329219).2068-83-9C06001440873373733-HYDROXY-ISOBUTYRATE389707C[C@@H](CO)C(O)=OC4H8O3InChI=1S/C4H8O3/c1-3(2-5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1DBXBTMSZEOQQDU-VKHMYHEASA-N(2S)-3-hydroxy-2-methylpropanoic acid104.1045104.0473441220.742(S)-3-hydroxyisobutyric acid0-1FDB021877(s)-3-hydroxyisobutyric acid;2-methyl-l-(+)-hydracrylate;2-methyl-l-(+)-hydracrylic acid;3-hydroxy(iso)butyric acid;3-hydroxy-2-methyl-(s)-propanoate;3-hydroxy-2-methyl-(s)-propanoic acid;3-hydroxy-2-methylpropanoate;3-hydroxy-2-methylpropanoic acid;3-hydroxy-isobutyrate;3-hydroxyisobutyrate;3-hydroxyisobutyric acid;(s)-3-hydroxy-2-methylpropanoic acid;(s)-3-hydroxy-2-methylpropionic acid;(s)-3-hydroxy-2-methylpropanoate;(s)-3-hydroxyisobutyrate;(s)-3-hydroxy-2-methylpropionatePW_C0000163-HIBA4878167339082226791941121215684071241251191500(S)-Methylmalonic acid semialdehydeHMDB0002217Methylmalonic semialdehyde is a metabolite in valine catabolism, inositol metabolism and propanoate metabolism. Methylmalonate-semialdehyde dehydrogenase (MMSDH) catalyses the NAD+ and coenzyme A-dependent conversion of methylmalonate semialdehyde to propionyl-CoA in the distal region of the L-valine catabolic pathway. MMSDH is located within the mitochondria; direct enzymatic assay of MMSDH is difficult since the substrate, methylmalonate semialdehyde, is both commercially unavailable and notoriously unstable as a b-keto acid. (PMID: 10947204).99043-16-0C060025462303278214575365C[C@@H](C=O)C(O)=OC4H6O3InChI=1S/C4H6O3/c1-3(2-5)4(6)7/h2-3H,1H3,(H,6,7)/t3-/m0/s1VOKUMXABRRXHAR-VKHMYHEASA-N(2S)-2-methyl-3-oxopropanoic acid102.0886102.0316940580.421(S)-methylmalonaldehydic acid0-1FDB022912(2s)-2-methyl-3-oxopropanoate;(2s)-2-methyl-3-oxopropanoic acid;(s)-methylmalonate semialdehyde;(s)-methylmalonic acid semialdehydePW_C00150022M3O167532314490872247862613379195112121570407122255406124127119124808120126417479127983501130Methylmalonic acidHMDB0000202Methylmalonic acid is a malonic acid derivative, which is a vital intermediate in the metabolism of fat and protein. In particular, the coenzyme A-linked form of methylmalonic acid, methylmalonyl-CoA, is converted into succinyl-CoA by methylmalonyl-CoA mutase in a reaction that requires vitamin B12 as a cofactor. In this way, methylmalonic acid enters the Krebs cycle and is thus part of one of the anaplerotic reactions. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This inborn error of metabolism is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. Methylmalonic acid is also found to be associated with other inborn errors of metabolism, including cobalamin deficiency, cobalamin malabsorption, malonyl-CoA decarboxylase deficiency, and transcobalamin II deficiency. When present in sufficiently high levels, methylmalonic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of methylmalonic acid are associated with at least 5 inborn errors of metabolism, including Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders. Methylmalonic acid is an organic acid and abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.516-05-2C0217048730860473DB04183CC(C(O)=O)C(O)=OC4H6O4InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)ZIYVHBGGAOATLY-UHFFFAOYSA-N2-methylpropanedioic acid118.088118.026608680.102methylmalonic acid0-2FDB0219051,1-ethanedicarboxylate;1,1-ethanedicarboxylic acid;2-methylmalonate;2-methylmalonic acid;Isosuccinate;Isosuccinic acid;Methyl-malonate;Methyl-malonic acid;Methyl-propanedioate;Methyl-propanedioic acid;Methylmalonate;Methylmalonic acid;Methylpropanedioate;Methylpropanedioic acid;Alpha-methylmalonic acid;2-methylpropanedioate;A-methylmalonate;A-methylmalonic acid;Alpha-methylmalonate;α-methylmalonate;α-methylmalonic acidPW_C000130Isosca16838231644565184567245691578627133792051117922913079231132792321148267780121287125121289430121584122122256406123858136123860465124142135124809120126418479127984501988Propionyl-CoAHMDB0001275Propionyl-CoA is an intermediate in the metabolism of propanoate. Propionic aciduria is caused by an autosomal recessive disorder of propionyl coenzyme A (CoA) carboxylase deficiency (EC 6.4.1.3). In propionic aciduria, propionyl CoA accumulates within the mitochondria in massive quantities; free carnitine is then esterified, creating propionyl carnitine, which is then excreted in the urine. Because the supply of carnitine in the diet and from synthesis is limited, such patients readily develop carnitine deficiency as a result of the increased loss of acylcarnitine derivatives. This condition demands supplementation of free carnitine above the normal dietary intake to continue to remove (detoxify) the accumulating organic acids. Propionyl-CoA is a substrate for Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial), Acetyl-coenzyme A synthetase 2-like (mitochondrial), Propionyl-CoA carboxylase alpha chain (mitochondrial), Methylmalonate-semialdehyde dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal), Acyl-CoA dehydrogenase (long-chain specific, mitochondrial), Malonyl-CoA decarboxylase (mitochondrial), Acetyl-coenzyme A synthetase (cytoplasmic), 3-ketoacyl-CoA thiolase (mitochondrial) and Propionyl-CoA carboxylase beta chain (mitochondrial). (PMID: 10650319).317-66-8C0010043916415539PROPIONYL-COA388310CCC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC24H40N7O17P3SInChI=1S/C24H40N7O17P3S/c1-4-15(33)52-8-7-26-14(32)5-6-27-22(36)19(35)24(2,3)10-45-51(42,43)48-50(40,41)44-9-13-18(47-49(37,38)39)17(34)23(46-13)31-12-30-16-20(25)28-11-29-21(16)31/h11-13,17-19,23,34-35H,4-10H2,1-3H3,(H,26,32)(H,27,36)(H,40,41)(H,42,43)(H2,25,28,29)(H2,37,38,39)/t13-,17-,18-,19?,23-/m1/s1QAQREVBBADEHPA-UXYNFSPESA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy({3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy})phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid823.597823.141423115-2.169[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB0225292-methylacetyl-coa;2-methylacetyl-coenzyme a;Propanoyl-coa;Propanoyl-coenzyme a;Propionyl-coa;Propionyl-coenzyme a;Alpha-methylacetyl-coa;Alpha-methylacetyl-coenzyme aPW_C000988PropCoA12778169432285424455491413909122477641334784361127855611178636133120995122121576407121681408122266406123560135124133119124231374124819120125935297126430479126557482126568481127395205127997501128130502128141206112L-IsoleucineHMDB0000172Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)-tyrosine, tryptophan and phenylalanine, as well as methionine-are increased in these conditions. Valine, in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. The BCAA are not without side effects. Leucine alone, for example, exacerbates pellagra and can cause psychosis in pellagra patients by increasing excretion of niacin in the urine. Leucine may lower brain serotonin and dopamine. A dose of 3 g of isoleucine added to the niacin regime has cleared leucine-aggravated psychosis in schizophrenic patients. Isoleucine may have potential as an antipsychotic treatment. Leucine is more highly concentrated in foods than other amino acids. A cup of milk contains 800 mg of leucine and only 500 mg of isoleucine and valine. A cup of wheat germ has about 1.6 g of leucine and 1 g of isoleucine and valine. The ratio evens out in eggs and cheese. One egg and an ounce of most cheeses each contain about 400 mg of leucine and 400 mg of valine and isoleucine. The ratio of leucine to other BCAA is greatest in pork, where leucine is 7 to 8 g and the other BCAA together are only 3 to 4 g. (http://www.dcnutrition.com).73-32-5C00407630617191ILE6067DB00167CC[C@H](C)[C@H](N)C(O)=OC6H13NO2InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1AGPKZVBTJJNPAG-WHFBIAKZSA-N(2S,3S)-2-amino-3-methylpentanoic acid131.1729131.094628665-0.062L-isoleucine00FDB012397(2s,3s)-2-amino-3-methylpentanoate;(2s,3s)-2-amino-3-methylpentanoic acid;(2s,3s)-2-amino-3-methyl-pentanoate;(2s,3s)-2-amino-3-methyl-pentanoic acid;(2s,3s)-a-amino-b-methyl-n-valerate;(2s,3s)-a-amino-b-methyl-n-valeric acid;(2s,3s)-a-amino-b-methylvalerate;(2s,3s)-a-amino-b-methylvaleric acid;(2s,3s)-alph-amino-beta-methylvalerate;(2s,3s)-alph-amino-beta-methylvaleric acid;(2s,3s)-alpha-amino-beta-merthyl-n-valerate;(2s,3s)-alpha-amino-beta-merthyl-n-valeric acid;(2s,3s)-alpha-amino-beta-merthylvalerate;(2s,3s)-alpha-amino-beta-merthylvaleric acid;(2s,3s)-alpha-amino-beta-methyl-n-valerate;(2s,3s)-alpha-amino-beta-methyl-n-valeric acid;(2s,3s)-alpha-amino-beta-methylvalerate;(2s,3s)-alpha-amino-beta-methylvaleric acid;(s)-isoleucine;(s,s)-isoleucine;2-amino-3-methylpentanoate;2-amino-3-methylpentanoic acid;2-amino-3-methylvalerate;2-amino-3-methylvaleric acid;2s,3s-isoleucine;Erythro-l-isoleucine;Ile;Iso-leucine;Isoleucine;L-(+)-isoleucine;L-ile;[s-(r*,r*)]-2-amino-3-methylpentanoate;[s-(r*,r*)]-2-amino-3-methylpentanoic acid;Alpha-amino-beta-methylvaleric acid;I;A-amino-b-methylvalerate;A-amino-b-methylvaleric acid;Alpha-amino-beta-methylvalerate;α-amino-β-methylvalerate;α-amino-β-methylvaleric acidPW_C000112Ile1724856561075657108714718771481884254231542562318791831111215461221241041353733-Methyl-2-oxovaleric acidHMDB00004913-Methyl-2-oxovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 3-Methyl-2-oxovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 3-methyl-2-oxovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465). 3-Methyl-2-oxovaleric acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development.1460-34-0C0346547359322-KETO-3-METHYL-VALERATE46CCC(C)C(=O)C(O)=OC6H10O3InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)JVQYSWDUAOAHFM-UHFFFAOYSA-N3-methyl-2-oxopentanoic acid130.1418130.062994186-1.1213-methyl-2-oxopentanoic acid0-1FDB021447(3r)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoic acid;(r)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoic acid;(s)-3-methyl-2-oxopentanoate;(s)-3-methyl-2-oxopentanoic acid;2-keto-3-methylvalerate;2-keto-3-methylvaleric acid;2-oxo-3-methyl-n-valerate;2-oxo-3-methyl-n-valeric acid;2-oxo-3-methylpentanoate;2-oxo-3-methylpentanoic acid;2-oxo-3-methylvalerate;2-oxo-3-methylvaleric acid;2-oxoisoleucine;2-oxokolavenate;2-oxokolavenic acid;3-methyl-2-oxo-valerate;3-methyl-2-oxo-valeric acid;3-methyl-2-oxo-pentanoate;3-methyl-2-oxo-pentanoic acid;3-methyl-2-oxopentanoate;3-methyl-2-oxopentanoic acid;3-methyl-2-oxovalerate;3-methyl-2-oxovaleric;A-keto-b-methyl-n-valerate;A-keto-b-methyl-n-valeric acid;A-keto-b-methylvalerate;A-keto-b-methylvaleric acid;A-oxo-b-methyl-n-valerate;A-oxo-b-methyl-n-valeric acid;A-oxo-b-methylvalerate;A-oxo-b-methylvaleric acid;Alpha-keto-beta-methyl-n-valerate;Alpha-keto-beta-methyl-n-valeric acid;Alpha-keto-beta-methylvalerate;Alpha-keto-beta-methylvaleric acid;Alpha-oxo-beta-methyl-n-valerate;Alpha-oxo-beta-methyl-n-valeric acid;Alpha-oxo-beta-methylvalerate;Alpha-oxo-beta-methylvaleric acid;3-ethyl-3-methylpyruvic acid;3-ethyl-3-methylpyruvate;α-keto-β-methyl-n-valerate;α-keto-β-methyl-n-valeric acid;α-keto-β-methylvalerate;α-keto-β-methylvaleric acid;α-oxo-β-methyl-n-valerate;α-oxo-β-methyl-n-valeric acid;α-oxo-β-methylvalerate;α-oxo-β-methylvaleric acidPW_C0003733M2Oxva172586244151791841111215471221241051352980S-(2-Methylbutanoyl)-dihydrolipoamideHMDB0006869S-(2-Methylbutanoyl)-dihydrolipoamide-E is an intermediate in isoleucine degradation. S-(2-Methylbutanoyl)-dihydrolipoamide is normally conjugated to a lysine residue of the methylpropanoyltransferase enzyme (E stands for enzyme). The structure shown here is the free form. Specifically S-(2-Methylbutanoyl)-dihydrolipoamide-E is the 2-methylbutanoyl thioester of the reduced lipoyllysine residue in dihydrolipoyllysine-residue (2-methylpropanoyl)transferase.C0511844056528692389465CCC(C)C(=O)SCCC(S)CCCCC(N)=OC13H25NO2S2InChI=1S/C13H25NO2S2/c1-3-10(2)13(16)18-9-8-11(17)6-4-5-7-12(14)15/h10-11,17H,3-9H2,1-2H3,(H2,14,15)UFNCWFSSEGPJNL-UHFFFAOYSA-N8-[(2-methylbutanoyl)sulfanyl]-6-sulfanyloctanamide291.473291.132670429-4.4428-[(2-methylbutanoyl)sulfanyl]-6-sulfanyloctanamide00FDB024125S-(2-methylbutanoyl)dihydrolipoyllysine;[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase];S-(2-methylbutanoyl)-dihydrolipoamide;S-(8-amino-8-oxo-3-sulfanyloctyl) 2-methylbutanethioic acid;S-(8-amino-8-oxo-3-sulphanyloctyl) 2-methylbutanethioate;S-(8-amino-8-oxo-3-sulphanyloctyl) 2-methylbutanethioic acidPW_C002980S2MD1666417352791741321215361241240941188202-Methylbutyryl-CoAHMDB00010412-Methylbutyryl-CoA is a a product of isoleucine catabolism. It is converted to Tiglyl-CoA by short/branched-chain acyl-CoA dehydrogenase. 2-Methylbutyryl-CoA dehydrogenase deficiency, also called 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency or MBHD, is an inherited disorder in which the body is unable to process the amino acid isoleucine properly. It is caused by a mutation in the HADH2 gene. Untreated MBHD can lead to progressive loss of motor skills, to mental retardation and to epilepsy. 2-Methylbutyryl-CoA is a substrate for Acyl-CoA dehydrogenase (short-chain specific, mitochondrial), Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial) and Acyl-CoA dehydrogenase (long-chain specific, mitochondrial).6712-02-3C01033439371154772-METHYL-BUTYRYL-COA388491CCC(C)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC26H44N7O17P3SInChI=1S/C26H44N7O17P3S/c1-5-14(2)25(38)54-9-8-28-16(34)6-7-29-23(37)20(36)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-19(49-51(39,40)41)18(35)24(48-15)33-13-32-17-21(27)30-12-31-22(17)33/h12-15,18-20,24,35-36H,5-11H2,1-4H3,(H,28,34)(H,29,37)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41)/t14?,15-,18-,19-,20?,24-/m1/s1LYNVNYDEQMMNMZ-PCLZRLGGSA-N4-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)-2-hydroxy-3,3-dimethyl-N-[2-({2-[(2-methylbutanoyl)sulfanyl]ethyl}-C-hydroxycarbonimidoyl)ethyl]butanimidic acid851.651851.172723243-2.179α-methylbutyryl-coenzyme A0-4FDB022387(s)-2-methylbutyryl-coa;(s)-2-methylbutyryl-coenzyme a;2-methylbutanoyl-coa;2-methylbutanoyl-coenzyme a;2-methylbutyryl-coa;2-methylbutyryl-coenzyme a;3'-phosphoadenosine 5'-{3-[(3r)-3-hydroxy-2,2-dimethyl-4-{[3-({2-[(2-methylbutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl] dihydrogen diphosphate};Methylbutanoyl-coa;Methylbutanoyl-coenzyme a;Alpha-methylbutyryl-coa;Alpha-methylbutyryl-coenzyme a;A-methylbutyryl-coaPW_C0008202IbuCoA1667417463791911121215634071241211191378Tiglyl-CoAHMDB0002054Tiglyl-CoA is a metabolite in the degradation of isoleucine to propionic acid pathway. A defect in the conversion of tiglyl-CoA to alpha-methyl-beta-hydroxybutyryl-CoA, results in episodic abdominal pain and acidosis in patients with Tiglic acidemia (OMIM 275190).C03345528056415478TIGLYL-COA4444187C\C=C(/C)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C26H42N7O17P3SInChI=1S/C26H42N7O17P3S/c1-5-14(2)25(38)54-9-8-28-16(34)6-7-29-23(37)20(36)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-19(49-51(39,40)41)18(35)24(48-15)33-13-32-17-21(27)30-12-31-22(17)33/h5,12-13,15,18-20,24,35-36H,6-11H2,1-4H3,(H,28,34)(H,29,37)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41)/b14-5+/t15-,18-,19-,20?,24-/m1/s1PMWATMXOQQZNBX-APMDNKNFSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(2E)-2-methylbut-2-enoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid849.635849.157073179-2.199[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(2E)-2-methylbut-2-enoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB022819(e)-2-methylcrotonoyl-coa;(e)-2-methylcrotonoyl-coenzyme a;2-methylbut-2-enoyl-coa;2-methylbut-2-enoyl-coenzyme a;2-methylcrotanoyl-coa;2-methylcrotanoyl-coenzyme a;2-methylcrotonoyl-coa;2-methylcrotonoyl-coenzyme a;2-methylcrotonyl-coa;2-methylcrotonyl-coenzyme a;Methylcrotonoyl-coa;Methylcrotonoyl-coenzyme a;Methylcrotonyl-coa;Methylcrotonyl-coenzyme a;Tigloyl-coa;Tigloyl-coenzyme a;Tiglyl-coa;Tiglyl-coenzyme a;Trans-2-methylbut-2-enoyl-coa;Trans-2-methylbut-2-enoyl-coenzyme aPW_C001378Tig-CoA175047919713312157340612413012010472-Methyl-3-hydroxybutyryl-CoAHMDB00013562-Methyl-3-hydroxybutyryl-CoA is a substrate for 3-hydroxyacyl-CoA dehydrogenase type II, Enoyl-CoA hydratase (mitochondrial), Trifunctional enzyme alpha subunit (mitochondrial), Short chain 3-hydroxyacyl-CoA dehydrogenase (mitochondrial) and Peroxisomal bifunctional enzyme.52227-66-4C04405440326154492-METHYL-3-HYDROXY-BUTYRYL-COA389295C[C@H](O)[C@H](C)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC26H44N7O18P3SInChI=1S/C26H44N7O18P3S/c1-13(14(2)34)25(39)55-8-7-28-16(35)5-6-29-23(38)20(37)26(3,4)10-48-54(45,46)51-53(43,44)47-9-15-19(50-52(40,41)42)18(36)24(49-15)33-12-32-17-21(27)30-11-31-22(17)33/h11-15,18-20,24,34,36-37H,5-10H2,1-4H3,(H,28,35)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t13-,14-,15+,18+,19+,20?,24+/m0/s1PEKYNTFSOBAABV-SYASONGASA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-3-({2-[(2-{[(2S,3S)-3-hydroxy-2-methylbutanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)-2,2-dimethylpropoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid867.65867.167637865-2.1910[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-3-({2-[(2-{[(2S,3S)-3-hydroxy-2-methylbutanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)-2,2-dimethylpropoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB022575(2s,3s)-3-hydroxy-2-methylbutanoyl-coa;(2s,3s)-3-hydroxy-2-methylbutanoyl-coenzyme a;(2s,3s)-3-hydroxy-2-methylbutyryl-coa;(2s,3s)-3-hydroxy-2-methylbutyryl-coenzyme a;(s)-3-hydroxy-2-methylbutyryl-coa;(s)-3-hydroxy-2-methylbutyryl-coenzyme a;2-methyl-3-hydroxybutyryl-coa;2-methyl-3-hydroxybutyryl-coenzyme a;3-hydroxy-2-methylbutyryl-coa;3-hydroxy-2-methylbutyryl-coenzyme aPW_C0010472M3HC17514791981331215744061241311208972-Methylacetoacetyl-CoAHMDB00011572-Methylacetoacetyl-CoA is a substrate for 3-hydroxyacyl-CoA dehydrogenase type II, 3-ketoacyl-CoA thiolase (mitochondrial), Peroxisomal bifunctional enzyme, Trifunctional enzyme beta subunit (mitochondrial), Short chain 3-hydroxyacyl-CoA dehydrogenase (mitochondrial) and 3-ketoacyl-CoA thiolase (peroxisomal).6712-01-2C0334453154762-METHYL-ACETO-ACETYL-COA52CC(C(C)=O)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C26H42N7O18P3SInChI=1S/C26H42N7O18P3S/c1-13(14(2)34)25(39)55-8-7-28-16(35)5-6-29-23(38)20(37)26(3,4)10-48-54(45,46)51-53(43,44)47-9-15-19(50-52(40,41)42)18(36)24(49-15)33-12-32-17-21(27)30-11-31-22(17)33/h11-13,15,18-20,24,36-37H,5-10H2,1-4H3,(H,28,35)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)NHNODHRSCRALBF-UHFFFAOYSA-N{[5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(2-methyl-3-oxobutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid865.634865.151987801-2.2092-methylacetoacetyl-coa0-4FDB0224552-methyl-3-acetoacetyl-coa;2-methyl-3-acetoacetyl-coenzyme a;2-methylacetoacetyl-coa;2-methylacetoacetyl-coenzyme a;S-(2-methyl-3-oxobutanoate;S-(2-methyl-3-oxobutanoate) coa;S-(2-methyl-3-oxobutanoate) coenzyme a;S-(2-methyl-3-oxobutanoic acid;Alpha-methylacetoacetyl-coa;Alpha-methylacetoacetyl-coenzyme aPW_C0008972Ma-CoA1752379199112121575407124132119134Oxoglutaric acidHMDB0000208Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422011863198126812897705425377135133774811117752311277746129779673457797034677976327779843477842533480018368806941351131629411997240612002212412008440712017412212055241412081441812098940812114642312115242412116042512275712012283111912318645012339945412355437412371845812372445912373246012535747912540029912545548112553329712580048912592948212690050112694038812699320612706620512725550612738850295L-Glutamic acidHMDB0000148Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeurePW_C000095Glu16244365811911384164149699110542144850145626146254532311153441135415117543911855651325631107563210858591056006147607115761919465318568381876844188709272709371716520571822077514224751815182082258373220117921981185516112004222126213112683289126972904234831542349318428453207702025377332133775251127797134677977327779813477829134580649135120023124120040122120086407120347406120692126120816418121147423121153424121157425122833119122997120123299443123401454123719458123725459123729460125401299125418297125457481125667479125769301125802489126941388126995206127162501127257506140738841407395971188PyridoxalHMDB0001545Pyridoxal, also known as pyridoxaldehyde, belongs to the class of organic compounds known as pyridoxals and derivatives. Pyridoxals and derivatives are compounds containing a pyridoxal moiety, which consists of a pyridine ring substituted at positions 2,3,4, and 5 by a methyl group, a hydroxyl group, a carbaldehyde group, and a hydroxymethyl group, respectively. Pyridoxal exists as a solid, soluble (in water), and a very weakly acidic compound (based on its pKa). Pyridoxal has been found in human kidney and placenta tissues, and has also been primarily detected in blood. Within the cell, pyridoxal is primarily located in the cytoplasm. Pyridoxal exists in all living organisms, ranging from bacteria to humans. Pyridoxal can be converted into pyridoxal 5'-phosphate; which is catalyzed by the enzyme pyridoxal kinase. In humans, pyridoxal is involved in the vitamin B6 metabolism pathway and the valine, leucine and isoleucine degradation pathway. Pyridoxal is also involved in several metabolic disorders, some of which include Beta-ketothiolase deficiency, the 3-methylglutaconic aciduria type IV pathway, the methylmalonic aciduria pathway, and isobutyryl-CoA dehydrogenase deficiency. The 4-carboxyaldehyde form of vitamin B6 which is converted to pyridoxal phosphate which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid.66-72-8C00250105017310PYRIDOXAL1021DB00147CC1=NC=C(CO)C(C=O)=C1OC8H9NO3InChI=1S/C8H9NO3/c1-5-8(12)7(4-11)6(3-10)2-9-5/h2,4,10,12H,3H2,1H3RADKZDMFGJYCBB-UHFFFAOYSA-N3-hydroxy-5-(hydroxymethyl)-2-methylpyridine-4-carbaldehyde167.162167.058243159-1.162pyridoxal00FDB0111693-hydroxy-5-(hydroxymethyl)-2-methylpyridine-4-carboxaldehyde;Pyridoxal;Pyridoxaldehyde;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehydePW_C001188Pyridox15842165381681157703222579180132792111141215421241219284091241001181244811371260972991261014831276833881276852081316Carbon dioxideHMDB0001967Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-Nmethanedione44.009543.9898292440.630carbon dioxide00DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981851148Pyridoxal 5'-phosphateHMDB0001491This is the active form of vitamin B6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (pyridoxamine). -- Pubchem; Pyridoxal-phosphate (PLP, pyridoxal-5'-phosphate) is a cofactor of many enzymatic reactions. It is the active form of vitamin B6 which comprises three natural organic compounds, pyridoxal, pyridoxamine and pyridoxine. -- Wikipedia.54-47-7C00018105118405PYRIDOXAL_PHOSPHATE1022DB00114CC1=NC=C(COP(O)(O)=O)C(C=O)=C1OC8H10NO6PInChI=1S/C8H10NO6P/c1-5-8(11)7(3-10)6(2-9-5)4-15-16(12,13)14/h2-3,11H,4H2,1H3,(H2,12,13,14)NGVDGCNFYWLIFO-UHFFFAOYSA-N[(4-formyl-5-hydroxy-6-methylpyridin-3-yl)methoxy]phosphonic acid247.1419247.024573569-1.643pyridoxal phosphate0-2FDB021820Apolon b6;Biosechs;Codecarboxylase;Coenzyme b6;Hairoxal;Hexermin-p;Hi-pyridoxin;Hiadelon;Himitan;Pal-p;Plp;Phosphopyridoxal;Phosphopyridoxal coenzyme;Pidopidon;Piodel;Pydoxal;Pyridoxal 5'-phosphate;Pyridoxal 5-phosphate;Pyridoxal p;Pyridoxal phosphate;Pyridoxal-p;Pyridoxyl phosphate;Pyromijin;Sechvitan;Vitahexin-p;Vitazechs;3-hydroxy-2-methyl-5-[(phosphonooxy)methyl]-4-pyridinecarboxaldehyde;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphate;Phosphoric acid mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphoric acid ester;Pyridoxal 5'-(dihydrogen phosphate);Pyridoxal-5'-phosphate;Pyridoxal 5'-phosphoric acid;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphoric acid;Phosphate mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphate ester;Pyridoxal 5'-(dihydrogen phosphoric acid);Pyridoxal 5-phosphoric acid;Pyridoxal phosphoric acid;Pyridoxal-5'-phosphoric acidPW_C001148Pyr-5'P182324453518122140119696201110421450501458262120102150495325111541611754211035441118545512055671325581133653385701816071672057216212722221311858161121751511262331126281812684289126892907701725377037225770412937705222477526112777643417797334677979327782923457885533278862331806961359863071199121221200241241200294061200874071208174181211494231211554241220691231220763831228341191234024541237214581237274591246204471246273981253022971254022991254074791254584811258034891262242981262314951269423881269475011269962061272585061277865131277933901099Coenzyme AHMDB0001423Coenzyme A (CoA, CoASH, or HSCoA) is a coenzyme notable for its role in the synthesis and oxidization of fatty acids and the oxidation of pyruvate in the citric acid cycle. It is adapted from beta-mercaptoethylamine, panthothenate, and adenosine triphosphate. It is also a parent compound for other transformation products, including but not limited to, phenylglyoxylyl-CoA, tetracosanoyl-CoA, and 6-hydroxyhex-3-enoyl-CoA. Coenzyme A is synthesized in a five-step process from pantothenate and cysteine. In the first step pantothenate (vitamin B5) is phosphorylated to 4'-phosphopantothenate by the enzyme pantothenate kinase (PanK, CoaA, CoaX). In the second step, a cysteine is added to 4'-phosphopantothenate by the enzyme phosphopantothenoylcysteine synthetase (PPC-DC, CoaB) to form 4'-phospho-N-pantothenoylcysteine (PPC). In the third step, PPC is decarboxylated to 4'-phosphopantetheine by phosphopantothenoylcysteine decarboxylase (CoaC). In the fourth step, 4'-phosphopantetheine is adenylylated to form dephospho-CoA by the enzyme phosphopantetheine adenylyl transferase (CoaD). Finally, dephospho-CoA is phosphorylated using ATP to coenzyme A by the enzyme dephosphocoenzyme A kinase (CoaE). Since coenzyme A is, in chemical terms, a thiol, it can react with carboxylic acids to form thioesters, thus functioning as an acyl group carrier. CoA assists in transferring fatty acids from the cytoplasm to the mitochondria. A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. When it is not attached to an acyl group, it is usually referred to as 'CoASH' or 'HSCoA'. Coenzyme A is also the source of the phosphopantetheine group that is added as a prosthetic group to proteins such as acyl carrier proteins and formyltetrahydrofolate dehydrogenase. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA which is a vital component in cholesterol and ketone synthesis. Furthermore, it contributes an acetyl group to choline to produce acetylcholine in a reaction catalysed by choline acetyltransferase. Its main task is conveying the carbon atoms within the acetyl group to the citric acid cycle to be oxidized for energy production (Wikipedia).85-61-0C0001068161146900CO-A6557CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCSC21H36N7O16P3SInChI=1S/C21H36N7O16P3S/c1-21(2,16(31)19(32)24-4-3-12(29)23-5-6-48)8-41-47(38,39)44-46(36,37)40-7-11-15(43-45(33,34)35)14(30)20(42-11)28-10-27-13-17(22)25-9-26-18(13)28/h9-11,14-16,20,30-31,48H,3-8H2,1-2H3,(H,23,29)(H,24,32)(H,36,37)(H,38,39)(H2,22,25,26)(H2,33,34,35)/t11-,14-,15-,16+,20-/m1/s1RGJOEKWQDUBAIZ-IBOSZNHHSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-({2-[(2-sulfanylethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid767.534767.115208365-2.2210coenzyme A0-4FDB022614Acetoacetyl coenzyme a sodium salt;Coa;Coa hydrate;Coa-sh;Coash;Coenzyme a;Coenzyme a hydrate;Coenzyme a-sh;Coenzyme ash;Coenzymes a;Depot-zeel;Propionyl coa;Propionyl coenzyme a;S-propanoate;S-propanoate coa;S-propanoate coenzyme a;S-propanoic acid;S-propionate coa;S-propionate coenzyme a;Zeel;[(2r,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-4-hydroxy-3-(phosphonooxy)tetrahydrofuran-2-yl]methyl 3-hydroxy-4-({3-oxo-3-[(2-sulfanylethyl)amino]propyl}amino)-2,2-dimethyl-4-oxobutyl dihydrogen diphosphatePW_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678918511Lipoyl-GMPHMDB0013617Lipoyl-GMP is an intermediate in lipoic acid metabolism.In mammals, the posttranslational modification of unlipoylated apoproteins by addition of lipoic acid is done in a two-step enzymatic reaction using free lipoic acid. The free lipoic acid in the cell is first activated by reacting it. with adenosine triphosphate, using up two high energy phosphate bonds to form lipoyl-AMP (GMP), a process catalyzed by lipoate activating enzyme (LAE).41 The activated lipoyl moiety is subsequently used to form an amide bond with lysine residues of the unlipoylated apoprotein, which is catalyzed by lipoyl-AMP(GMP):N-lysine lipoyl transferase Interestingly, purified LAE from bovine liver mitochondria preferentially utilizes guanosine triphosphate, instead of adenosine triphosphate, for the activation of lipoic acid to form lipoyl-GMP.44 Furthermore, genetic analysis of LAE reveals that the enzyme is identical to. xenobiotic-metabolizing/medium-chain fatty acid:CoA ligase-III (XL-III) (also from bovine liver mitochondria). 44 Hence, there appear to be two pathways in which LAE is capable of activating carboxylic acids: one that uses guanosine triphosphate to form acyl-GMP; the other is dependent on adenosine triphosphate for the activation of carboxylic acids to the CoA thioester (acyl-CoA) via an. acyl-AMP intermediate.53481910O[C@@H]1[C@@H](COP(O)(O)=O)O[C@H]([C@@H]1O)N1C=NC2=C1N=C(NC(=O)CCCCC1CCSS1)NC2=OC18H26N5O9PS2InChI=1S/C18H26N5O9PS2/c24-11(4-2-1-3-9-5-6-34-35-9)20-18-21-15-12(16(27)22-18)19-8-23(15)17-14(26)13(25)10(32-17)7-31-33(28,29)30/h8-10,13-14,17,25-26H,1-7H2,(H2,28,29,30)(H2,20,21,22,24,27)/t9?,10-,13-,14-,17-/m1/s1DVZGUOFXTLKZTJ-LQLSJNDNSA-N{[(2R,3S,4R,5R)-5-{2-[5-(1,2-dithiolan-3-yl)pentanamido]-6-oxo-6,9-dihydro-1H-purin-9-yl}-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid551.531551.090955347-2.696[(2R,3S,4R,5R)-5-{2-[5-(1,2-dithiolan-3-yl)pentanamido]-6-oxo-1H-purin-9-yl}-3,4-dihydroxyoxolan-2-yl]methoxyphosphonic acid0-2PW_C008511L-GMP1596417473435827918611212155140712410911929813-Hydroxyisovaleryl-CoAHMDB00068703-Hydroxyisovaleryl-CoA belongs to the class of organic compounds known as (s)-3-hydroxyacyl coas. These are organic compounds containing a (S)-3-hydroxyl acylated coenzyme A derivative. 3-Hydroxyisovaleryl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). 3-Hydroxyisovaleryl-CoA has been primarily detected in urine. Within the cell, 3-hydroxyisovaleryl-CoA is primarily located in the cytoplasm. 3-Hydroxyisovaleryl-CoA exists in all living organisms, ranging from bacteria to humans. In humans, 3-hydroxyisovaleryl-CoA is involved in the valine, leucine and isoleucine degradation pathway. 3-Hydroxyisovaleryl-CoA is also involved in several metabolic disorders, some of which include the 3-methylglutaconic aciduria type III pathway, the isovaleric acidemia pathway, 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, and 2-methyl-3-hydroxybutryl CoA dehydrogenase deficiency. 3-Hydroxyisovaleryl-CoA is an end product of leucine degradation. It is converted from 3-methylbut-2-enoyl-CoA by the enzyme enoyl-CoA hydratase.C05998119538762829110128175CC(C)(O)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC26H44N7O18P3SInChI=1S/C26H44N7O18P3S/c1-25(2,20(37)23(38)29-6-5-15(34)28-7-8-55-16(35)9-26(3,4)39)11-48-54(45,46)51-53(43,44)47-10-14-19(50-52(40,41)42)18(36)24(49-14)33-13-32-17-21(27)30-12-31-22(17)33/h12-14,18-20,24,36-37,39H,5-11H2,1-4H3,(H,28,34)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t14-,18-,19-,20?,24-/m1/s1PEVZKILCBDEOBT-XBTRWLRFSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-3-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid867.65867.167637865-2.3110[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-3-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB0241263-hydroxy-3-methylbutanoyl-coa;3-hydroxy-3-methylbutanoyl-coenzyme a;3-hydroxy-3-methylbutyryl-coa;3-hydroxy-3-methylbutyryl-coenzyme a;3-hydroxyisovaleryl coenzyme aPW_C002981HIV-CoA1602443553791881121215564071241141191420WaterHMDB0002111Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71% of Earth's surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the body's solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the sun's energy to split off water's hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the sun's energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia).7732-18-5C0000196215377937OH2OInChI=1S/H2O/h1H2XLYOFNOQVPJJNP-UHFFFAOYSA-Nwater18.015318.0105646861water00FDB013390Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;WasserPW_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513140674790140675834140755185414Adenosine triphosphateHMDB0000538Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).56-65-5C00002595715422ATP5742DB00171NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H16N5O13P3InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1ZKHQWZAMYRWXGA-KQYNXXCUSA-N({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid507.181506.995745159-2.057adenosine triphosphate0-3FDB0218135'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acidPW_C000414ATP92214608266164142247813733327995934399763210518211210214649215614216058240559243427272646281229302966316372361661361751439923447431476891486454503289503526515575205975215100525010452911015313111534611253901035406117543011854431205542129555613255691335603135562110858461435854146587610758971475924151604815561091616230166649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862251184727711903170120102811203916412178285125782261269129013264223153273084232631542621322426943187702825377218134772333297746833377632336780373327804135078168128782143517824035378411335784941157885013078865331789193348002836880046184806741198562919482612411323494113282388116280109119914122119992406120154407120245382120362412121246429121392123121397433121471408121974410122065125122079383122083405122402422122444435122919399123009446123816464123951447123956468124029374124527444124616136124630398124634376124943472124972375125011470125304297125371479125392299125515481125595484126123485126220300126234495126240478126547491126596499126913501127123389127731516127781395127796390127801209128119508128167517140770891463Hydrogen carbonateHMDB0000595Bicarbonate, or hydrogen carbonate, is a simple single carbon molecule that plays surprisingly important roles in diverse biological processes. Among these are photosynthesis, the Krebs cycle, whole-body and cellular pH regulation, and volume regulation. Since bicarbonate is charged it is not permeable to lipid bilayers. Mammalian membranes thus contain bicarbonate transport proteins to facilitate the specific transmembrane movement of HCO3(-). Bicarbonate ion is an anion that consists of one central carbon atom surrounded by three oxygen atoms in a trigonal planar arrangement, with a hydrogen atom attached to one of the oxygens. The bicarbonate ion carries a negative one formal charge and is the conjugate base of carbonic acid, H2CO3. The carbonate radical is an elusive and strong one-electron oxidant. Bicarbonate in equilibrium with carbon dioxide constitutes the main physiological buffer. The bicarbonate-carbon dioxide pair stimulates the oxidation, peroxidation and nitration of several biological targets. The demonstration that the carbonate radical existed as an independent species in aqueous solutions at physiological pH and temperature renewed the interest in the pathophysiological roles of this radical and related species. The carbonate radical has been proposed to be a key mediator of the oxidative damage resulting from peroxynitrite production, xanthine oxidase turnover and superoxide dismutase1 peroxidase activity. The carbonate radical has also been proposed to be responsible for the stimulatory effects of the bicarbonate-carbon dioxide pair on oxidations mediated by hydrogen peroxide/transition metal ions. The ultimate precursor of the carbonate radical anion being bicarbonate, carbon dioxide, peroxymonocarbonate or complexes of transition metal ions with bicarbonate-derived species remains a matter of debate. The carbonate radical mediates some of the pathogenic effects of peroxynitrite. The carbonate radical as the oxidant produced from superoxide dismutase (EC 1.15.1.1, SOD1) peroxidase activity. Peroxymonocarbonate is a biological oxidant, whose existence is in equilibrium with hydrogen peroxide and bicarbonate. (PMID: 17505962, 17215880).71-52-3C0028876917544HCO3749OC(O)=OCH2O3InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4)BVKZGUZCCUSVTD-UHFFFAOYSA-Ncarbonic acid62.024862.000393930.572carbonic acid0-1FDB022134Bicarbonate;Bicarbonate (hco3-);Bicarbonate anion;Bicarbonate ion;Bicarbonate ion (hco31-);Bicarbonate ions;Carbonate;Carbonate (hco31-);Carbonate ion (hco31-);Carbonic acid;Hydrocarbonate(1-);Hydrogen carbonate;Hydrogen carbonate (hco3-);Hydrogen carbonate anion;Hydrogen carbonate ion;Hydrogen carbonate ion (hco3-);Hydrogencarbonate;Hydrogentrioxocarbonate;Monohydrogen carbonate;[co2(oh)](-);Acid carbonate;Hco3(-);Hydrogen carbonic acid;Acid carbonic acid;Bicarbonic acid;Bicarbonic acid ionPW_C000463HCO322416878239332397226131531457053911035445120557113360491556110161649417874822229092224779591127863013278762111800293681199934061212094071214361221215571241237791191239941351241151181253724791260592971263602991265414811269145011275112051279223881281142061034Adenosine diphosphateHMDB0001341Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.58-64-0C00008602216761ADP5800NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H15N5O10P2InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1XTWYTFMLZFPYCI-KQYNXXCUSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid427.2011427.029414749-2.126adenosine-diphosphate0-2FDB021817Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acidPW_C001034ADP23413484152248213801596315978310611415182190149210418211310216158240859243527272847273646285529316572363561440023447631477091503626515775208975217100531511153491125392103544612055441295572133562410857411175764101584914358561465878107589914759261516050155611116162311666495178670094684118868721607159205718720672082107226213723121173001987303216739121774102187433163748322281872251185127711905170120132811218028513262223153293084232831542398313426223224269631877029253770871327721613477306329774723337766333678039332780433507817012878215351782443537841433578495115787053317884913078920334800303688062211880651135806761199482712411328338811620410911994412211999440612015640712031838212036641212124842912139412312139943312147240812189938312197641012206412512208540512240542212244543512297339912301344612381846412395344712395846812403037412445239812452944412461513612463637612494747212497537512501247012533429712537347912549229912551748112564548412612548512621930012623549512624247812655049112659749912691550112773351612778039512779739012780320912812250812816851712831338920BiotinHMDB0000030Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).58-85-5C0012017154815956BIOTIN149962DB00121[H][C@]12CS[C@@H](CCCCC(O)=O)[C@@]1([H])NC(=O)N2C10H16N2O3SInChI=1S/C10H16N2O3S/c13-8(14)4-2-1-3-7-9-6(5-16-7)11-10(15)12-9/h6-7,9H,1-5H2,(H,13,14)(H2,11,12,15)/t6-,7-,9-/m0/s1YBJHBAHKTGYVGT-ZKWXMUAHSA-N5-[(3aS,4S,6aR)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoic acid244.311244.088163078-2.3035-[(3aS,4S,6aR)-2-oxo-hexahydrothieno[3,4-d]imidazol-4-yl]pentanoic acid0-1FDB014510(+)-biotin;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valerate;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valeric acid;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valerate;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valeric acid;-(+)-biotin;1swk;1swn;1swr;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoate;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoic acid;Biodermatin;Bioepiderm;Bios ii;Bios h;Biotin;Coenzyme r;D(+)-biotin;D-(+)-biotin;D-biotin;D-biotin factor s;Factor s;Factor s (vitamin);Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoate;Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Lutavit h2;Meribin;Rovimix h 2;Vitamin b7;Vitamin h;Vitamin-h;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valerate;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valeric acid;Cis-hexahydro-2-oxo-1h-thieno(3,4)imidazole-4-valeric acid;Cis-tetrahydro-2-oxothieno(3,4-d)imidazoline-4-valeric acid;Delta-(+)-biotin;Delta-biotin;Delta-biotin factor s;Biotina;Biotine;BiotinumPW_C000020Biotin2641358579151699322702529210152981055393103544912055461115551114557513360511556112161649617869251607484222778311327796011280031368806531351199954061201341221205034091212104071215591241231091371237801191241171181253744791255012971257184831264212991265424811269165011270382051279893881281152061455(S)-beta-Aminoisobutyric acidHMDB0002166beta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. The concentration of beta-aminoisobutyric acid is normally low in urine as beta-aminoisobutyric acid is further catabolized by beta-aminoisobutyrate aminotransferases to methylmalonic acid semialdehyde and propionyl-CoA. beta-Aminoisobutyric acid occurs in two isomeric forms and both enantiomers of beta-aminoisobutyric acid can be detected in human urine and plasma. In plasma, the S-enantiomer is the predominant type due to active renal reabsorption. In contrast, urine almost exclusively contains the R-enantiomer of beta-aminoisobutyric acid, which is eliminated both by filtration and tubular secretion. Persistently increased levels of beta-aminoisobutyric acid have been observed in individuals with a deficiency of R (-)-beta-aminoisobutyrate-pyruvate aminotransferase. In addition, transient high levels of beta-aminoisobutyric acid have been observed under a variety of pathological conditions such as lead poisoning, starvation, in total body irradiation, and in a number of malignancies. The S-enantiomer of beta-aminoisobutyric acid is predominantly derived from the catabolism of valine. It has been suggested that altered homeostasis of beta-alanine underlies some of the clinical abnormalities encountered in patients with a dihydropyrimidine dehydrogenase (DPD) deficiency. DPD constitutes the first step of the pyrimidine degradation pathway, in which the pyrimidine bases uracil and thymine are catabolized to beta-alanine and the R-enantiomer of beta-aminoisobutyric acid respectively. In normal individuals with an intact pyrimidine degradation pathway, R-methylmalonic acid semialdehyde can be synthesized directly from the catabolism of thymine. Hence, there might be less cross-over between the valine and thymine pathway, allowing the conversion of S-methylmalonic acid semialdehyde into S-beta-aminoisobutyric acid and the subsequent accumulation of S-beta-aminoisobutyric acid in plasma (PMID: 14705962, 14292857, 14453202).4249-19-8C0328443943433094388543C[C@@H](CN)C(O)=OC4H9NO2InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1QCHPKSFMDHPSNR-VKHMYHEASA-N(2S)-3-amino-2-methylpropanoic acid103.1198103.0633285370.552L-β-aminoisobutyric acid00FDB022878(+)-a-methyl-b-alanine;(+)-alpha-methyl-beta-alanine;(+)-b-aminoisobutyric acid;(+)-beta-aminoisobutyric acid;(s)-3-amino-2-methylpropanoate;(s)-3-amino-2-methylpropanoic acid;(s)-3-amino-isobutanoate;(s)-3-amino-isobutanoic acid;(s)-3-amino-isobutyrate;(s)-3-amino-isobutyric acid;(s)-3-amino-2-methyl-propanoate;(s)-3-amino-2-methyl-propanoic acid;(s)-b-aminoisobutyric acid;(s)-beta-aminoisobutyric acid;L-2-methyl-b-alanine;L-2-methyl-beta-alanine;L-3-amino-2-methylpropanoate;L-3-amino-2-methylpropanoic acid;L-3-amino-2-methylpropionic acid;L-3-amino-isobutanoate;L-3-amino-isobutanoic acid;L-3-amino-isobutyrate;L-3-amino-isobutyric acid;L-b-aminoisobutyrate;L-b-aminoisobutyric acid;L-beta-aminoisobutyrate;L-beta-aminoisobutyric acid;S-b-aminoisobutyrate;S-beta-aminoisobutyrate;S-beta-aminoisobutyric acid;(s)-3-aminoisobutyrate;(s)-b-aminoisobutyrate;(s)-beta-aminoisobutyrate;(s)-β-aminoisobutyrate;(s)-β-aminoisobutyric acidPW_C001455L2MBA16824437939089151791961121215724071241291191560S-Methylmalonyl-CoAHMDB0002310S-Methylmalonyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. S-Methylmalonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). S-Methylmalonyl-CoA has been primarily detected in urine. Within the cell, S-methylmalonyl-CoA is primarily located in the cytoplasm, mitochondria and peroxisome. In humans, S-methylmalonyl-CoA is involved in the threonine and 2-oxobutanoate degradation pathway, the propanoate metabolism pathway, and the valine, leucine and isoleucine degradation pathway. S-Methylmalonyl-CoA is also involved in several metabolic disorders, some of which include the isovaleric aciduria pathway, the maple syrup urine disease pathway, isobutyryl-CoA dehydrogenase deficiency, and the 3-methylglutaconic aciduria type III pathway. Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).73173-91-8C0068321252287D-METHYL-MALONYL-COA13628334C[C@@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13?,16+,17+,18-,22?/m0/s1MZFOKIKEPGUZEN-JDVCRUKVSA-N(2S)-3-[(2-{3-[(2R)-3-[({[({[(3S,4R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid867.607867.131252359-2.3910(2S)-3-[(2-{3-[(2R)-3-{[({[(3S,4R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid0-5FDB022959(s)-methylmalonyl-coa;(s)-methylmalonyl-coenzyme aPW_C001560S-MmCoA228642695378623133790321121215784071222514061241351191248041201264144791267304811279795011283222061104PhosphateHMDB0001429Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.14265-44-2C00009106118367CPD-85871032OP(O)(O)=OH3O4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)NBIIXXVUZAFLBC-UHFFFAOYSA-Nphosphoric acid97.995297.9768950963phosphoric acid0-2DBMET00532FDB022617Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ionPW_C001104Pi24484881458181883129803176314176749250010272947273746312929316672363661385123424922447531503127515875207975216100531711153511125381103544712055431295573133560513556251085693658481435855146591114759411516040155610016162941076487178669110167141176842188688916071612057189206721221173061987389210740221274361637475222819622582582271011824110134257117481321176111511773213119041701192716412014281127282901326322334819174225530442350315424353184369232277018253771942937721713477940336779661307804833278057329782453537866933180022368892793089383138394796384110558390110640391113235941158453981162061091199824061200691221206994071210571241212161251212684291213521211214091231214233821218524051233041191236211181237861361238384641239684471239813991244053761249484721253624791254462971257744811259542991262214781265943001266042981267234841269045011274133881277832091281663951281775131283153891523R-Methylmalonyl-CoAHMDB0002255R-Methylmalonyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. R-Methylmalonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). R-Methylmalonyl-CoA has been primarily detected in urine. Within the cell, R-methylmalonyl-CoA is primarily located in the cytoplasm. In humans, R-methylmalonyl-CoA is involved in the threonine and 2-oxobutanoate degradation pathway, the valine, leucine and isoleucine degradation pathway, and the propanoate metabolism pathway. R-Methylmalonyl-CoA is also involved in several metabolic disorders, some of which include the maple syrup urine disease pathway, the 3-methylglutaconic aciduria type IV pathway, 3-methylcrotonyl CoA carboxylase deficiency type I, and 3-hydroxyisobutyric acid dehydrogenase deficiency. Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).73173-92-9 C01213 22833590METHYL-MALONYL-COA17216177C[C@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13?,16?,17?,18+,22?/m1/s1MZFOKIKEPGUZEN-YLYUOEEYSA-N(2R)-3-[(2-{3-[(2R)-3-[({[({[5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid867.607867.131252359-2.3910(2R)-3-[(2-{3-[(2R)-3-{[({[5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid0-5FDB022930PW_C001523R-MmCoA229042697243873786241337903513279204112121580407122252406122578124124138119124805120125150118126415479126733299127980501128326388808Succinyl-CoAHMDB0001022Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203).604-98-8C00091439161153803-METHYLBENZYLSUCCINYL-COA388307CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=OC25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20?,24-/m1/s1VNOYUJKHFWYWIR-FZEDXVDRSA-N4-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-4-oxobutanoic acid867.607867.131252359-2.19104-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-4-oxobutanoic acid0-5FDB022375Coa s-(hydrogen succinate);Coa s-succinate;Coenzyme a s-(hydrogen succinate);Coenzyme a s-succinate;S-(hydrogen butanedioate;S-(hydrogen butanedioate) coa;S-(hydrogen butanedioate) coenzyme a;S-(hydrogen butanedioic acid;S-succinoylcoenzyme a;Suc-co-a;Suc-coa;Succ-coa;Succ-coenzyme a;Succ-s-coa;Succ-s-coenzyme a;Succ-s-coenzyme-a;Succ-coenzyme-a;Succino-1-yl-coenzyme a;Succinyl coa;Succinyl coenzyme a;Succinyl-s-coa;Succinyl-s-coenzyme a;Succinyl-s-coenzyme-a;Succinylcoenzyme-a;Succinylcoenzyme aPW_C000808Suc-CoA233410553366925378103603915560971616485178701516073611637474222771401337810111278576132800213681199784061207694071220141241227631201233651191245681181253584791261642991263064811269015011278682061401AdenosylcobalaminHMDB0002086Adenosylcobalamin is one of two metabolically active forms synthesized upon ingestion of vitamin B12 and is the predominant form in the liver; it acts as a coenzyme in the reaction catalyzed by methylmalonyl-CoA mutase. A cobalamin (cbl) derivative in which the substituent is deoxyadenosyl. It is one of two metabolically active forms synthesized upon ingestion of vitamin B12 and is the predominant form in the liver; it acts as a coenzyme in the reaction catalyzed by methylmalonyl-CoA mutase (MCM; E.C. 5.4.99.2). Inborn errors of vitamin B12 metabolism are autosomal recessive disorders and have been classified into nine distinct complementation classes. Disorders affecting adenosylcobalamin cause methylmalonic acidemia and metabolic acidosis. Methylmalonyl-CoA mutase catalyzes the conversion of L-methylmalonyl-CoA to succinyl-CoA and uses adenosylcobalamin (AdoCbl) as a cofactor. Cbl must be transported into mitochondria, reduced and adenosylated before it can be utilized by MCM. (PMID: 17011224).13870-90-1C0019418408ADENOSYLCOBALAMIN-5-P30791458[C@H]1(C[Co-3]2345[N+]6=C7C(C)=C8N2[C@]([H])([C@H](CC(=O)N)[C@]8(CCC(=O)NC[C@@H](C)OP([O-])(=O)O[C@@H]2[C@@H](CO)O[C@H](N8C=[N+]3C3=CC(C)=C(C)C=C83)[C@@H]2O)C)[C@@]2(C)[C@@](CC(=O)N)([C@H](CCC(=O)N)C(C(C)=C3[N+]4=C(C=C6C([C@@H]7CCC(=O)N)(C)C)[C@@H](CCC(=O)N)[C@@]3(CC(=O)N)C)=[N+]52)C)O[C@@H](N2C3=NC=NC(=C3N=C2)N)[C@H](O)[C@@H]1OC72H100CoN18O17PInChI=1S/C62H90N13O14P.C10H12N5O3.Co/c1-29-20-39-40(21-30(29)2)75(28-70-39)57-52(84)53(41(27-76)87-57)89-90(85,86)88-31(3)26-69-49(83)18-19-59(8)37(22-46(66)80)56-62(11)61(10,25-48(68)82)36(14-17-45(65)79)51(74-62)33(5)55-60(9,24-47(67)81)34(12-15-43(63)77)38(71-55)23-42-58(6,7)35(13-16-44(64)78)50(72-42)32(4)54(59)73-56;1-4-6(16)7(17)10(18-4)15-3-14-5-8(11)12-2-13-9(5)15;/h20-21,23,28,31,34-37,41,52-53,56-57,76,84H,12-19,22,24-27H2,1-11H3,(H15,63,64,65,66,67,68,69,71,72,73,74,77,78,79,80,81,82,83,85,86);2-4,6-7,10,16-17H,1H2,(H2,11,12,13);/q;;+2/p-2/t31-,34-,35-,36-,37+,41-,52-,53-,56-,57+,59-,60+,61+,62+;4-,6-,7-,10-;/m11./s1ZIHHMGTYZOSFRC-OUCXYWSSSA-L(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-1-{[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-46-hydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate1579.58181578.6583455712(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-1-{[(2S,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-46-hydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate00FDB022837(5'-deoxy-5'-adenosyl)cobamide coenzyme;5'-deoxy-5'-adenosyl vitamin b12;5'-deoxy-5'-adenosylcobalamin;Adenosylcobalamin 5'-phosphate;Calomide;Cobalamin coenzyme;Cobamamide;Cobamamide 5'-phosphate;Cobamide coenzyme;Coenzyme b12;Deoxyadenosylcobalamin;Dibencozide;Funacomide;Vitamin b12 coenzyme;Vitamin b12 coenzymes;Adenosylcob(iii)alamin;5,6-dimethylbenzimidazolyl-5-deoxyadenosyl-cobamide;(5,6-dimethylbenzimidazolyl)cobamide coenzyme;Alpha-(5,6-dimethylbenzimidazolyl)cobamide coenzyme;5'-deoxy-5'-adenosyl-5,6-dimethylbenzimidazolylcobamide;5,6-dimethylbenzimidazolyl-co-5'-deoxy-5'-adenosylcobamide;Dmbc coenzyme;A-(5,6-dimethylbenzimidazolyl)cobamide coenzyme;α-(5,6-dimethylbenzimidazolyl)cobamide coenzymePW_C001401Adnscbn24013786281121215824071241401191264194811279852061065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-Ndioxygen31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O2959110524516500185058549146252863836491067431688207541576347693383621375492016242531222803294260424747135467123548012554931265508127580910859731476129159700618870321637050160731921375332107560212839515111816216118641981188321511894211120572251206316412247286122792261232524912706291127162921300429813016300130263011303830213260223422761742657315769102937704429477214134773501117736313077377331773953327749711377512115775373347762633677723337777361127774712977756341778051147781213378070329781511327838134578805343791113601200474081203831221204264051205424071205534141205944091206014061208834151210451241211043831216054341216564291221173821225734181226893841227983741228224431230271351230603761231284471231391361231634481231761191231874501232191371232261201234594511236091181236693981241634691242144641246693991251454541252751211254254821257064781257314831257372971257404791258844811261002991262724841265224951267214891268254801269645021269862071271982091272142081272192051272225011273055041273452061275573881275745151278353891280813951280953901283125061284323911783Hydrogen peroxideHMDB0003125Hydrogen peroxide (H2O2) is a very pale blue liquid which appears colourless in a dilute solution, slightly more viscous than water. It is a weak acid. It has strong oxidizing properties and is therefore a powerful bleaching agent that is mostly used for bleaching paper, but has also found use as a disinfectant and as an oxidizer. Hydrogen peroxide in the form of carbamide peroxide is widely used for tooth whitening (bleaching), both in professionally- and in self-administered products. Hydrogen peroxide (H2O2) is a well-documented component of living cells. It plays important roles in host defense and oxidative biosynthetic reactions. In addition there is growing evidence that at low levels, H2O2 also functions as a signaling agent, particularly in higher organisms. H2O2 has increasingly been viewed as an important cellular signaling agent in its own right, capable of modulating both contractile and growth-promoting pathways with more far-reaching effects. Due to the accumulation of hydrogen peroxide in the skin of patients with the depigmentation disorder vitiligo, the human epidermis cannot have the normal capacity for autocrine synthesis, transport and degradation of acetylcholine as well as the muscarinic (m1-m5) and nicotinic signal transduction in keratinocytes and melanocytes. Accumulating evidence suggests that hydrogen peroxide (H(2)O(2)) plays an important role in cancer development. Experimental data have shown that cancer cells produce high amounts of H(2)O(2). An increase in the cellular levels of H(2)O(2) has been linked to several key alterations in cancer, including DNA alterations, cell proliferation, apoptosis resistance, metastasis, angiogenesis and hypoxia-inducible factor 1 (HIF-1) activation. (PMID: 17150302, 17335854, 16677071, 16607324, 16514169).7722-84-1C0002778416240HYDROGEN-PEROXIDE763OOH2O2InChI=1S/H2O2/c1-2/h1-2HMHAJPDPJQMAIIY-UHFFFAOYSA-Nperoxol34.014734.0054793082hydrogen peroxide00FDB014562Adeka super el;Albone;Albone 35;Albone ds;Anti-keim 50;Asepticper;Baquashock;Cix;Clarigel gold;Crestal whitestrips;Crystacide;Dentasept;Deslime lp;Hioxyl;Hipox;Hybrite;Hydrogen dioxide;Hydrogen peroxide;Inhibine;Lase peroxide;Lensan a;Magic bleaching;Metrokur;Mirasept;Nite white excel 2;Odosat d;Opalescence xtra;Oxigenal;Oxydol;Oxyfull;Oxysept;Oxysept i;Pegasyl;Perhydrol;Perone;Peroxaan;Peroxclean;Quasar brite;Select bleach;Superoxol;T-stuff;Whiteness hp;Whitespeed;Xtra white;[oh(oh)];Dihydrogen dioxide;H2o2;HoohPW_C001783H2O298911351888551146272875515124331691217495125342238181047491347523154951265502123551012758101086005147703816383961511181721611886215124612261270929112719292130283011303529813040302134052224265831577022225770472947707929377500113775403347759811577720332777253377780611477810111778193267807332978152132785981121200504081201021221204634051205954091206094161209544071210471241221203821228013741228144431228391351230973761231574471231654481232201371232344521235201191236111181246723991254284821254692971257094781257324831257484881258954811261032991262754841269675021269782071270062051272012091272152081272305051273562061276013881278383891491MolybdopterinHMDB0002206Molybdenum cofactor is a cofactor required for the activity of enzymes such as sulfite oxidase, xanthine oxidoreductase, and aldehyde oxidase. It is a coordination complex formed between molybdopterin (which, despite the name, does not contain molybdenum) and an oxide of molybdenum. Molybdopterins, in turn, are synthesized from guanosine triphosphate. Molybdenum cofactor functions directly in ethylbenzene dehydrogenase, glyceraldehyde-3-phosphate ferredoxin oxidoreductase, and respiratory arsenate reductase. In animals and plants these enzymes use molybdenum bound at the active site in a tricyclic molybdenum cofactor. All molybdenum-using enzymes so far identified in nature use this cofactor The simplest structure of molybdopterin contains a pyranopterin coordinated to molybdenum. The pyranopterin structure is a fused ring system containing a pyran fused to pterin. In addition, the pyran ring is substituted with two thiols and an alkyl phosphate. In molybdopterin, the thiols coordinate to molybdenum. In some cases, the alkyl phosphate group is replaced by an alkyl diphosphate nucleotide. -- Wikipedia.73508-07-3C059242330423721437CPD0-18824883416O.O.[Mo++].[H]C1(COP(O)([O-])=O)OC2([H])NC3=C(NC2([H])C(S)=C1S)C([O-])=NC(=N)N3C10H16MoN5O8PS2InChI=1S/C10H14N5O6PS2.Mo.2H2O/c11-10-14-7-4(8(16)15-10)12-3-6(24)5(23)2(21-9(3)13-7)1-20-22(17,18)19;;;/h2-3,9,12,23-24H,1H2,(H2,17,18,19)(H4,11,13,14,15,16);;2*1H2/q;+2;;/p-2VUKICSJFFDCESC-UHFFFAOYSA-Lmolybdenum(2+) ion 8-[(hydrogen phosphonooxy)methyl]-2-imino-6,7-disulfanyl-1H,2H,5H,5aH,8H,9aH,10H-pyrano[3,2-g]pteridin-4-olate dihydrate525.31526.923197-2.747molybdenum(2+) ion 8-[(hydrogen phosphonooxy)methyl]-2-imino-6,7-disulfanyl-1H,5H,5aH,8H,9aH,10H-pyrano[3,2-g]pteridin-4-olate dihydrate0-2FDB022906Moco;Molybdenum cofactor;Molybdenum enzyme molybdenum cofactor;Molybdoenzyme molybdenum-containing cofactor;Nitrate reductase molybdenum cofactor;Pterin molybdenum cofactorPW_C001491mlyBdpn16848170423918263953294388346275475331483094838285498126551312712038151130313011304330277716113777283377838513279206112121585407121794124123153443123168448124143119124345118126108299127679388405582Fe-2SHMDB0061344Bis(λ²-iron(2+) ion) disulfane tetrasulfanide belongs to the class of inorganic compounds known as transition metal sulfides. These are inorganic compounds containing a sulfur atom of an oxidation state of -2, in which the heaviest atom bonded to the oxygen is a transition metal.S.S.[SH-].[SH-].[SH-].[SH-].[Fe++].[Fe++]Fe2H8S6InChI=1S/2Fe.6H2S/h;;6*1H2/q2*+2;;;;;;/p-4MZMMVZPHZTYDNI-UHFFFAOYSA-Jbis(lambda2-iron(2+) ion) disulfane tetrasulfanide312.11311.7648990bis(lambda2-iron(2+) ion) disulfane tetrasulfanide02PW_C0405582Fe2S37422401284257174389346265475431482994837285056454971265512127704616013030301130423027771511377727337783771347838613279207112117811133121586407121795124122567384122664406123152443123167448124144119124346118125140121125239120126189299126715480126796479127680388128306391128394501174Succinic acidHMDB0000254Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021).110-15-6C00042111015741SUC1078DB00139OC(=O)CCC(O)=OC4H6O4InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)KDYFGRWQOYBRFD-UHFFFAOYSA-Nbutanedioic acid118.088118.026608680.252succinic acid0-2FDB0019311,2-ethanedicarboxylate;1,2-ethanedicarboxylic acid;1,4-butanedioate;1,4-butanedioic acid;Amber acid;Asuccin;Dihydrofumarate;Dihydrofumaric acid;Katasuccin;Succinate;Wormwood acid;Acide butanedioique;Acide succinique;Acidum succinicum;Bernsteinsaeure;Butandisaeure;Butanedionic acid;E363;Ethylenesuccinic acid;Hooc-ch2-ch2-cooh;Spirit of amber;Butanedionate;EthylenesuccinatePW_C000174Succini1523239450218507867631126554255175383103604215561021616454107645510864891786764117683616673621637455219745622074772221186619812142151132592234236831842369315424023227714313377213134774831117773811277749129784263348002436880721119112846308113428111998440612019240712038512212055541412099040812256538412276712012302913512318945012355537412513812112536447912554948112593048212671348012690650112708220612738950212830439169electron-transfer flavoproteinCompoundPW_EC0000695086ChEBIETF70Reduced electron-transfer flavoproteinCompoundPW_EC0000705086ChEBIRETF2932-oxoisovalerate dehydrogenase subunit beta, mitochondrialP21953
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00299BCKDHB6q14.1X5244611.2.4.41589417382269332942-oxoisovalerate dehydrogenase subunit alpha, mitochondrialP12694The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00300BCKDHA19q13.1-q13.2Z1409311.2.4.415884173722694352Dihydrolipoyl dehydrogenase, mitochondrialP09622Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
HMDBP00054DLD7q31-q32L1375711.8.1.421741080346708639411369657021421265414252510201425961209Hydroxymethylglutaryl-CoA lyase, mitochondrialP35914Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism.
HMDBP00009HMGCL1p36.1-p35AK31386914.1.3.460341054314098413295Branched-chain-amino-acid aminotransferase, cytosolicP54687Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
HMDBP00301BCAT112p12.1CR74930812.6.1.4215852165482232Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialP11182The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP03078DBT1p31J0320812.3.1.168159741748343592161Isovaleryl-CoA dehydrogenase, mitochondrialP26440HMDBP00166IVD15q14-q15AF19121611.3.8.4160144352349056101Medium-chain specific acyl-CoA dehydrogenase, mitochondrialP11310This enzyme is specific for acyl chain lengths of 4 to 16.
HMDBP00104ACADM1p31M9143211.3.8.789744353343543460925270103135630133137854713380Enoyl-CoA hydratase, mitochondrialP30084Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate.
HMDBP00388ECHS110q26.2-q26.3BT00712314.2.1.17591485332944174605252371031356231331438851181489Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrialQ96RQ3HMDBP00510MCCC13q27AK02305116.4.1.41605443562487Methylcrotonoyl-CoA carboxylase beta chain, mitochondrialQ9HCC0HMDBP00508MCCC25q12-q13AL07929816.4.1.41606443572100Short-chain specific acyl-CoA dehydrogenase, mitochondrialP16219HMDBP00103ACADS12q24.31U8399211.3.8.158944365352721031356321331496Short/branched chain specific acyl-CoA dehydrogenase, mitochondrialP45954Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
HMDBP01613ACADSB10q26.13AF26067811.3.8.59264436434882652711031356311332441Isobutyryl-CoA dehydrogenase, mitochondrialQ9UKU7Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex.
HMDBP05273ACAD811q25AF26068111.3.99.-167144363324253-hydroxyisobutyryl-CoA hydrolase, mitochondrialQ6NVY1Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
HMDBP04709HIBCH2q32.2AK22297913.1.2.41674322834460823873-hydroxyisobutyrate dehydrogenase, mitochondrialP31937HMDBP00395HIBADH7p15.2AC00509111.1.1.31167632424-aminobutyrate aminotransferase, mitochondrialP80404Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
HMDBP00248ABAT16p13.2U8022612.6.1.19; 2.6.1.2246314885424457523713-hydroxyacyl-CoA dehydrogenase type-2Q99714Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).
HMDBP00379HSD17B10Xp11.2BC00870811.1.1.35; 1.1.1.51; 1.1.1.1788493266944910614311610081438821181313-ketoacyl-CoA thiolase, mitochondrialP42765Abolishes BNIP3-mediated apoptosis and mitochondrial damage.
HMDBP00031ACAA218q21.1D1629412.3.1.1684639034135626133143887118149Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrialQ02252Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.
HMDBP00051ALDH6A114q24.3AJ24999411.2.1.18; 1.2.1.2754441695346132253Propionyl-CoA carboxylase alpha chain, mitochondrialP05165HMDBP00259PCCA13q32AY03579516.4.1.3170032287446142252Propionyl-CoA carboxylase beta chain, mitochondrialP05166HMDBP00258PCCB3q21-q22M3116716.4.1.317013461521428474250Methylmalonyl-CoA epimerase, mitochondrialQ96PE7HMDBP00256MCEE2p13.3AF36454715.1.99.1171432291426982251Methylmalonyl-CoA mutase, mitochondrialP22033Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species.
HMDBP00257MUT6p12.3M3750015.4.99.21715424023121Aldehyde oxidaseQ06278HMDBP00126AOX12q33CH47106311.2.3.1; 1.17.3.-168581705239192639542943903475531289Aldehyde dehydrogenase, mitochondrialP05091HMDBP00295ALDH212q24.2K0300111.2.1.354741321818713300921354921814157795614202197914347554920Methylglutaconyl-CoA hydratase, mitochondrialQ13825Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.
HMDBP00982AUH9q22.31AL51335314.2.1.181650338Hydroxymethylglutaryl-CoA synthase, mitochondrialP54868This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
HMDBP00040HMGCS21p13-p12U8185612.3.3.1059641052380333813784171336Acetyl-CoA acetyltransferase, mitochondrialP24752Plays a major role in ketone body metabolism.
HMDBP00038ACAT111q22.3CH47106512.3.1.95944105035281103135633133139651785140982131412892676Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrialP55809Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.
HMDBP00712OXCT15p13.1AB02957612.8.3.55984105634352-oxoisovalerate dehydrogenase subunit beta, mitochondrial1PW_P0004354582934342-oxoisovalerate dehydrogenase subunit alpha, mitochondrial1PW_P0004344572942041060156Dihydrolipoyl dehydrogenase E31PW_P000056595222996422104182Hydroxymethylglutaryl-CoA lyase, mitochondrial1PW_P00018220092433Branched-chain-amino-acid aminotransferase, cytosolic1PW_P0004334562952031188110602-oxoisovalerate dehydrogenase1PW_P001060120629411207293142911481436Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial1PW_P000436459223220885111437Isovaleryl-CoA dehydrogenase, mitochondrial1PW_P0004374601612099641251Medium-chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P00025127010141169641177Enoyl-CoA hydratase, mitochondrial1PW_P0001771953806438Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial1PW_P000438461489210201439Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial1PW_P000439462487176Short-chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P0001761941004809641252Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P000252271149641179641454Isobutyryl-CoA dehydrogenase, mitochondrial1PW_P000454477244121696414583-hydroxyisobutyryl-CoA hydrolase, mitochondrial1PW_P00045848124254593-hydroxyisobutyrate dehydrogenase, mitochondrial1PW_P00045948238764-aminobutyrate aminotransferase, mitochondrial1PW_P000006724227114814062423-hydroxyacyl-CoA dehydrogenase type-21PW_P00024226037144813-ketoacyl-CoA thiolase, mitochondrial1PW_P000481504314158Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial1PW_P0001581764945433463Propionyl-CoA carboxylase alpha chain, mitochondrial1PW_P00046348625322120116974464Propionyl-CoA carboxylase beta chain, mitochondrial1PW_P000464487252465Methylmalonyl-CoA epimerase, mitochondrial1PW_P000465488250468Methylmalonyl-CoA mutase, mitochondrial1PW_P000468491251228614011461Aldehyde oxidase1PW_P0004614841211218964121914911427405581159Aldehyde dehydrogenase, mitochondrial1PW_P00015917728945454449Methylglutaconyl-CoA hydratase, mitochondrial1PW_P000449472920180Hydroxymethylglutaryl-CoA synthase, mitochondrial1PW_P000180198382179Acetyl-CoA acetyltransferase, mitochondrial1PW_P000179197364450Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial1PW_P000450473676956falsePW_R000956Right377229761Compoundfalse37737691Compoundfalse377429781Compoundfalse5534341.2.4.45544351.2.4.4234falsePW_R000234Right10147821Compoundfalse10157211Compoundtrue10167691Compoundfalse101711441Compoundtrue1018400341Compoundtrue61561.8.1.4980falsePW_R000980Right385510631Compoundfalse38569401Compoundfalse5811824.1.3.4981falsePW_R000981Right385710631Compoundfalse3858421Compoundfalse5821824.1.3.4994falsePW_R000994Right389229761Compoundfalse389329771Compoundfalse389410601Compoundfalse6014341.2.4.46024351.2.4.4987falsePW_R000987Right387129771Compoundfalse387229791Compoundfalse5934341.2.4.45944351.2.4.41027falsePW_R001027Right39837691Compoundfalse398429771Compoundfalse398529801Compoundfalse398610601Compoundfalse6714341.2.4.46724351.2.4.41031falsePW_R001031Right39957691Compoundfalse399629791Compoundfalse6794341.2.4.46804351.2.4.4985falsePW_R000985Right38677041Compoundfalse78221341Compoundtrue3868121Compoundfalse7823951Compoundtrue5904332.6.1.42954falsePW_R000954Right37685401Compoundfalse78241341Compoundtrue37695471Compoundfalse7825951Compoundtrue5484332.6.1.421024falsePW_R001024Right39751121Compoundfalse78261341Compoundtrue39763731Compoundfalse7827951Compoundtrue6664332.6.1.42419falsePW_R000419Unknown17215471Compoundfalse172210601Compoundfalse172329761Compoundfalse172413161Compoundtrue206310601.2.4.4958falsePW_R000958Right377829781Compoundfalse782810991Compoundtrue37798701Compoundfalse37807821Compoundfalse5574362.3.1.168959falsePW_R000959Right37818701Compoundfalse7829691ElementCollectiontrue378211501Compoundfalse7830701ElementCollectiontrue5584371.3.8.4960falsePW_R000960Both37838701Compoundfalse7831691ElementCollectiontrue378411501Compoundfalse7832701ElementCollectiontrue5592511.3.8.7961falsePW_R000961Right378529811Compoundfalse378611501Compoundfalse783314201Compoundtrue5601774.2.1.17962falsePW_R000962Right378711501Compoundfalse78344141Compoundtrue78354631Compoundtrue37888321Compoundfalse783610341Compoundtrue5614386.4.1.45624396.4.1.41033falsePW_R001033Right399929791Compoundfalse783710991Compoundtrue40009601Compoundfalse40017821Compoundfalse6824362.3.1.168418falsePW_R000418Right1717121Compoundfalse171810601Compoundfalse171929771Compoundfalse172013161Compoundtrue206510601.2.4.42214falsePW_R002214Right78443731Compoundfalse784510601Compoundfalse784629771Compoundfalse784713161Compoundtrue206710601.2.4.42215falsePW_R002215Right784829801Compoundfalse784910991Compoundtrue78508201Compoundfalse78517821Compoundfalse20684362.3.1.1682217falsePW_R002217Right78569601Compoundfalse7857691ElementCollectiontrue78588001Compoundfalse7859701ElementCollectiontrue20721761.3.8.120732521.3.8.520744541.3.99.-1002falsePW_R001002Both39169601Compoundfalse7860691ElementCollectiontrue39178001Compoundfalse7861701ElementCollectiontrue6122511.3.8.71003falsePW_R001003Right39188001Compoundfalse786214201Compoundtrue39198291Compoundfalse6131774.2.1.171005falsePW_R001005Right39228291Compoundfalse786314201Compoundtrue3923161Compoundfalse786410991Compoundtrue6154583.1.2.461falsePW_R000061Right232161Compoundfalse2337211Compoundtrue23415001Compoundfalse23511441Compoundtrue236400341Compoundtrue20784591.1.1.3120791774.2.1.17274falsePW_R000274Both116214551Compoundfalse11631341Compoundtrue116415001Compoundfalse1165951Compoundtrue208061036falsePW_R001036Both40068201Compoundfalse7876691ElementCollectiontrue400713781Compoundfalse7877701ElementCollectiontrue6862511.3.8.71035falsePW_R001035Right40048201Compoundfalse7878691ElementCollectiontrue400513781Compoundfalse7879701ElementCollectiontrue6841761.3.8.16852521.3.8.51037falsePW_R001037Right400813781Compoundfalse788414201Compoundtrue400910471Compoundfalse6871774.2.1.171038falsePW_R001038Both401010471Compoundfalse78947211Compoundtrue40118971Compoundfalse789511441Compoundtrue6882421039falsePW_R001039Right40128971Compoundfalse789810991Compoundtrue40139881Compoundfalse40149401Compoundfalse6894812.3.1.161011falsePW_R001011Right393415001Compoundfalse790114201Compoundtrue79027211Compoundtrue790310991Compoundtrue39359881Compoundfalse790411441Compoundtrue79054631Compoundtrue6271581012falsePW_R001012Right39369881Compoundfalse79064141Compoundtrue79074631Compoundtrue393715601Compoundfalse790810341Compoundtrue790911041Compoundtrue6284636.4.1.36294646.4.1.31263falsePW_R001263Right482915601Compoundfalse483015231Compoundfalse9764655.1.99.11486falsePW_R001486Right563715231Compoundfalse56388081Compoundfalse12074685.4.99.21010falsePW_R001010Right393215001Compoundfalse791414201Compoundtrue791510651Compoundtrue39331301Compoundfalse791617831Compoundtrue6244611282falsePW_R001282Right490115001Compoundfalse79207211Compoundtrue792114201Compoundtrue49021301Compoundfalse792211441Compoundtrue20871591.2.1.32230PW_R002230Right79231301Compoundfalse792415231Compoundfalse979falsePW_R000979Right38538321Compoundfalse792714201Compoundtrue385410631Compoundfalse5804494.2.1.18682falsePW_R000682Right27949401Compoundfalse279511421Compoundfalse279614201Compoundtrue279710631Compoundfalse279810991Compoundtrue2089180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227 C127 177 177 127 227 127 C1620 127 3431 127 4824 127 C4874 127 4924 177 4924 227 C4924 1848 4924 3954 4924 5575 C4924 5625 4874 5675 4824 5675 C3431 5675 1620 5675 227 5675 C177 5675 127 5625 127 5575 C127 3954 127 1848 127 227 1true64797.05548.0475338M380 1271 C380 1221 430 1171 480 1171 C1751 1171 3404 1171 4675 1171 C4725 1171 4775 1221 4775 1271 C4775 2545 4775 4200 4775 5474 C4775 5524 4725 5574 4675 5574 C3404 5574 1751 5574 480 5574 C430 5574 380 5524 380 5474 C380 4200 380 2545 380 1271 84true64395.04403.0475339M470 1366 C470 1316 520 1266 570 1266 C1787 1266 3370 1266 4587 1266 C4637 1266 4687 1316 4687 1366 C4687 2588 4687 4178 4687 5400 C4687 5450 4637 5500 4587 5500 C3370 5500 1787 5500 570 5500 C520 5500 470 5450 470 5400 C470 4178 470 2588 470 1366 84true64217.04234.0890846235Mitochondria6431280201.61.620015890847235Cytosol453305202.52.5200156232718493971184475455644#FFEBEB443574380Isovaleric AciduriaIsovaleric acidemia (IVA) is caused by mutation in the isovaleryl CoA dehydrogenase gene. Isovaleryl CoA dehydrogenase is part of the acyl-CoA dehydrogenase family and is involved in the catabolism of leucine. A defect in this enzyme causes accumulation of ammonia, ketone bodies, Isovaleryl/2-Methylbutyrylcarnitine (C5) in blood; carnitine in plasma; creatinine, and glucose in serum; 3-Hydroxybutyric acid, 3-Hydroxyisovaleric acid, 4-Hydroxyvaleric acid, acetyltryptophan, glycine, acylcarnitin, isovalerylasparagine, isovalerylglycine, isovaleryllysine, isovalerylhistidine and isovaleryltryptophan in urine. Symptoms include encephalopathy, ketosis, metabolic acidosis, pancreatitis, sweaty feet odor, and thrombocytopenia.DiseasePW_X000105Context105466161ProteinMutated467870CompoundIncreased824[Uniprot: P26440](http://www.uniprot.org/uniprot/P26440)105Context825[OMIM: Entry 243500](http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=243500)105Context826Engelke, U., van der Graaf, M., Heerschap, A., Hoenderop, S., Moolenaar, S., Morava, E., Wevers, R. Handbook of 1H-NMR spectroscopy in inborn errors of metabolism: body fluid NMR spectroscopy and in vivo MR spectroscopy (2nd ed) (2007) p.58 Heilbronn: SPS Verlagsgesellschaft105Context8274684367Ando T, Nyhan WL, Bachmann C, Rasmussen K, Scott R, Smith EK: Isovaleric acidemia: identification of isovalerate, isovalerylglycine, and 3-hydroxyisovalerate in urine of a patient previously reported as having butyric and hexanoic acidemia. J Pediatr. 1973 Feb;82(2):243-8.105Context8286015910Efron ML: Isovaleric acidemia. Am J Dis Child. 1967 Jan;113(1):74-6.105Context82915486829Ensenauer R, Vockley J, Willard JM, Huey JC, Sass JO, Edland SD, Burton BK, Berry SA, Santer R, Grunert S, Koch HG, Marquardt I, Rinaldo P, Hahn S, Matern D: A common mutation is associated with a mild, potentially asymptomatic phenotype in patients with isovaleric acidemia diagnosed by newborn screening. Am J Hum Genet. 2004 Dec;75(6):1136-42. doi: 10.1086/426318. Epub 2004 Oct 14.105Context8302063866Vockley J, Parimoo B, Tanaka K: Molecular characterization of four different classes of mutations in the isovaleryl-CoA dehydrogenase gene responsible for isovaleric acidemia. Am J Hum Genet. 1991 Jul;49(1):147-57.105Context2785743385530Berry GT, Yudkoff M, Segal S: Isovaleric acidemia: medical and neurodevelopmental effects of long-term therapy. J Pediatr. 1988 Jul;113(1 Pt 1):58-64.105Context