PathWhiz

PathWhiz ID	Pathway	Meta Data
PW121869 View Pathway	disease 2-Ketoglutarate Dehydrogenase Complex Deficiency Mus musculus 2-Ketoglutarate dehydrogenase complex deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency or oxoglutaric aciduria, is an autosomal recessive disorder of the Krebs cycle caused by a defective oxoglutarate dehydrogenase complex (OGDC). OGDC catalyzes the conversion of 2-ketoglutarate into succinyl-CoA. This disorder is characterized by a large accumulation of 2-ketoglutarate in the urine. Symptoms of the disorder include opisthotonus, ataxia, developmental delay, and seizures. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:50 Last Updated: September 10, 2018 at 15:50
PW132375 View Pathway	metabolic 2-mercaptobenzothiazole Drug Metabolism Homo sapiens 2-mercaptobenzothiazole is a drug that is not metabolized by the human body as determined by current research and biotransformer analysis. 2-mercaptobenzothiazole passes through the liver and is then excreted from the body mainly through the kidney. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: September 21, 2023 at 21:19 Last Updated: September 21, 2023 at 21:19
PW146374 View Pathway	drug action 2-mercaptobenzothiazole Drug Metabolism Action Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 07, 2023 at 18:03 Last Updated: October 07, 2023 at 18:03
PW121916 View Pathway	disease 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency Rattus norvegicus 2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:50 Last Updated: September 10, 2018 at 15:50
PW121690 View Pathway	disease 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency Mus musculus 2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:49 Last Updated: September 10, 2018 at 15:49
PW000061 View Pathway	disease 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency Homo sapiens 2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: August 01, 2013 at 15:52 Last Updated: August 01, 2013 at 15:52
PW127223 View Pathway	disease 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency Homo sapiens 2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: November 15, 2022 at 11:02 Last Updated: November 15, 2022 at 11:02
PW002096 View Pathway	metabolic 2-O-alpha-Mannosyl-D-glycerate Degradation Escherichia coli 2-O-α-Mannosyl-D-glycerate (MG; also named as Alpha-Mannosylglycerate) is an organic compound that will affect the osmosis in hyperthermophilic archaea and bacteria. In E.coli, 2-O-α-mannosyl-D-glycerate PTS permease (mngA) import MG into cell, and then phosphorylate MG to 2-O-(6-phospho-α-mannosyl)-D-glycerate by phosphocarrier protein HPr. 2-O-(6-phospho-α-mannosyl)-D-glycerate is converted to glyceric acid as well as mannose 6-phosphate by alpha-mannosidase mngB. Finally, glyceric acid is catalyzed to 2-Phospho-D-glyceric acid with ATP as energy source by Glycerate kinase 2. E.coli can't use MG as osmotic stress protection, but it can use MG as a carbon source. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: October 13, 2015 at 17:28 Last Updated: October 13, 2015 at 17:28
PW002108 View Pathway	metabolic 2-Oxoglutarate Decarboxylation to Succinyl-CoA Escherichia coli 2-oxoglutarate dehydrogenase complex is consisted of oxoglutarate decarboxylase, dihydrolipoyl succinyltransferase and dihydrolipoyl dehydrogenase), which is a rate-limiting enzyme of the citric acid cycle (TCA cycle) in prokaryote. The reaction that catalyzed by 2-oxoglutarate dehydrogenase complex can be generalized as 2-oxoglutarate + coenzyme A + NAD+ â†’ succinyl-CoA + CO2 + NADH. During the OGDHC reaction cycle, 2-oxoglutarate is bound and decarboxylated by E1(o), a thiamin-diphosphate cofactor containing enzyme. The succinyl group is transferred to the lipoyl domain of E2(o) where it is carried to the active site and transferred to coenzyme A, forming succinyl-CoA. During this transfer the lipoyl group is reduced to dihydrolipoyl. The succinyl-CoA is released and the lipoyl domain of E2(o) is oxidized by E3 via transfer of protons to NAD, forming NADH and regenerating the lipoyl group back to lipoyllysine for another cycle. Under aerobic growth conditions the OGDHC not only catalyzes a key reaction in the TCA cycle, it also provides succinyl-CoA for methionine and lysine biosynthesis, the latter pathway also leading to peptidoglycan biosynthesis. The synthesis of the OGDHC is repressed by anaerobiosis and is also subject to glucose repression. It is induced by aerobic growth on acetate. (EcoCyc) BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: October 14, 2015 at 11:56 Last Updated: October 14, 2015 at 11:56
PW123572 View Pathway	metabolic 2-Oxoglutarate Decarboxylation to Succinyl-CoA Pseudomonas aeruginosa 2-oxoglutarate dehydrogenase complex is consisted of oxoglutarate decarboxylase, dihydrolipoyl succinyltransferase and dihydrolipoyl dehydrogenase), which is a rate-limiting enzyme of the citric acid cycle (TCA cycle) in prokaryote. The reaction that catalyzed by 2-oxoglutarate dehydrogenase complex can be generalized as 2-oxoglutarate + coenzyme A + NAD+ â†’ succinyl-CoA + CO2 + NADH. During the OGDHC reaction cycle, 2-oxoglutarate is bound and decarboxylated by E1(o), a thiamin-diphosphate cofactor containing enzyme. The succinyl group is transferred to the lipoyl domain of E2(o) where it is carried to the active site and transferred to coenzyme A, forming succinyl-CoA. During this transfer the lipoyl group is reduced to dihydrolipoyl. The succinyl-CoA is released and the lipoyl domain of E2(o) is oxidized by E3 via transfer of protons to NAD, forming NADH and regenerating the lipoyl group back to lipoyllysine for another cycle. Under aerobic growth conditions the OGDHC not only catalyzes a key reaction in the TCA cycle, it also provides succinyl-CoA for methionine and lysine biosynthesis, the latter pathway also leading to peptidoglycan biosynthesis. The synthesis of the OGDHC is repressed by anaerobiosis and is also subject to glucose repression. It is induced by aerobic growth on acetate. (EcoCyc) BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: August 12, 2019 at 22:33 Last Updated: August 12, 2019 at 22:33