Description

2-Methylacetoacetic acid is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase deficiency. Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1), or the peroxisomes (ACAA2). Thiolases are CoA-dependent enzymes which catalyze the formation of a carbon-carbon bond in a Claisen condensation step and its reverse reaction via a thiolytic degradation mechanism. Mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (T2) is important in the pathways for the synthesis and degradation of ketone bodies as well as for the degradation of 2-methylacetoacetyl-CoA. PMID: 17371050, PMID: 14518824, PMID: 9744475, PMID: 9001814 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-Methylacetoacetate

Basic Part Properties

Part Name
2-Methylacetoacetate
Part Display Name
2-Methylacetoacetate
Part Type
Component (Describes the core component or analyte measured)
Created On
2010-04-09
Construct for LOINC Short Name
2-Methylacetoacetate

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Language Variants Get Info

TagLanguageTranslation
zh-CNChinese (China)2-甲基乙酰乙酸乙酯
Synonyms: α-丁酮酸乙酯;Α-丁酮酸乙酯;甲基乙醯乙酸乙酯;Α-甲基乙酰乙酸乙酯;Α-甲基乙酰乙酸乙酯
fr-CAFrench (Canada)2-méthylacétoacetate
et-EEEstonian (Estonia)2-metüülatseetoatsetaat
es-ESSpanish (Spain)2-Metilacetoacetato
it-ITItalian (Italy)2-Metilacetoacetato
tr-TRTurkish (Turkey)2-Metilasetoasetat
ru-RURussian (Russian Federation)2-метилацетоацетат
nl-NLDutch (Netherlands)2-methylacetoacetaat
fr-BEFrench (Belgium)2-Methylacetoacetate
Synonyms: 2-Méthyl-acétoacétate
fr-FRFrench (France)2-méthylacétoacétate
pl-PLPolish (Poland)2-metyloacetooctan
el-GRGreek (Greece)2-Ακετοξικό μεθύλιο
Synonyms: 2-Ακετοξικό μεθύλιο