IUPAC 名称
{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-2-({[({[(3R)-3-{[2-({2-[(3E)-dec-3-enoylsulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid
描述
trans-3-Decenoyl-CoA is an intermediate in fatty acid metabolism. trans-3-Decenoyl-CoA is the substrate of medium-chain acyl-CoA dehydrogenase (MCAD, EC 1.3.99.3) MCAD acts on C4-C16 acyl-CoAs with its peak activity toward medium-chain (C6-C12) substrates. MCAD is a key enzyme for the beta-oxidation of fatty acids. MCAD deficiency is caused by mutation in the medium-chain acyl-CoA dehydrogenase gene (ACADM; OMIM 607008). Inherited deficiency of medium-chain acyl-CoA dehydrogenase is characterized by intolerance to prolonged fasting, recurrent episodes of hypoglycemic coma with medium-chain dicarboxylic aciduria, impaired ketogenesis, and low plasma and tissue carnitine levels. The disorder may be severe, and even fatal, in young patients. It has been reported that between 19 and 25% of patients with undiagnosed deficiency of MCAD die during their first episode of metabolic decompensation. (PMID: 15850406) [HMDB]
InChI标识符
InChI=1S/C31H52N7O17P3S/c1-4-5-6-7-8-9-10-11-22(40)59-15-14-33-21(39)12-13-34-29(43)26(42)31(2,3)17-52-58(49,50)55-57(47,48)51-16-20-25(54-56(44,45)46)24(41)30(53-20)38-19-37-23-27(32)35-18-36-28(23)38/h9-10,18-20,24-26,30,41-42H,4-8,11-17H2,1-3H3,(H,33,39)(H,34,43)(H,47,48)(H,49,50)(H2,32,35,36)(H2,44,45,46)/b10-9+/t20-,24-,25-,26+,30-/m1/s1