Accession NumberHMDB02095
Common_NameMalonylcarnitine
DescriptionMalonylcarnitine is a metabolite that accumulates with specific disruption of fatty-acid oxidation caused by impaired entry of long-chain acylcarnitine esters into the mitochondria and failure of the mitochondrial respiratory chain at complex 11 and malonyl-CoA decarboxylase (EC 4.1.1.9) deficiency (OMIM 248360). Malonylcarnitine has also been found to accumulate in some newborns with medium-chain acyl-CoA dehydrogenase (EC 1.3.99.3) deficiency (OMIM 201450). (PMID 11558490, 15303003, 12651823)
Chemical_IUPAC_Name(3S)-3-(3-hydroxy-3-oxopropanoyl)oxy-4-trimethylazaniumylbutanoate
Chemical FormulaC10H17NO6
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