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3-Methyl-2-oxovaleric acid is a metabolite of isoleucine in man, animals and bacteria. It is the alpha-keto acid analogue of isoleucine. 3-Methyl-2-oxovaleric acid is produced from isoleucine by cytosolic branched chain aminotransferase 1 (EC:, whereupon it is further degraded by branched chain keto acid dehydrogenase E1 to 2-Methyl-1-hydroxybutyl-ThPP. It is used as a clinical marker for maple syrup urine disease (MSUD). MSUD is caused by a deficiency of the branched-chain -keto acid dehydrogenase compex resulting in an accumulation of branched-chain amino acids and the corresponding -keto-and -hydroxy acids in blood, urine and cerebrospinal fluid causing neurological damage and mental retardation. Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.) Source: Human Metabolome Database, 2-Oxo,3-Methylvalerate

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Tag Language Translation
zh-CN Chinese (China) 2-氧代,3-甲基戊酸
Synonyms: 2Oxo3Me-戊酸;2-酮-3-甲基戊酸;2-酮基-3-甲基戊酸
fr-CA French (Canada) 2-oxo,3-méthylvalérate
es-ES Spanish (Spain) 2-Oxo,3-Metilvalerato
it-IT Italian (Italy) 2-Osso,3-Metilvalerato
tr-TR Turkish (Turkey) 2-Okzo,3-Metilvalerat
ru-RU Russian (Russian Federation) 2-Оксо,3-Метилвалерат
nl-NL Dutch (Netherlands) 2-oxo,3-methylvaleraat
fr-BE French (Belgium) 2-oxo,3-méthylvalérate