Classification Term: 170048

脂肪酸酯 (ontology term: 5f421870922794aba64f704a506f06a6)

found 1 associated metabolites at sub_class metabolite taxonomy ontology rank level.

Ancestor: 脂质和类脂分子

Child Taxonomies: There is no child term of current ontology term.

Malonylcarnitine

(3R)-3-[(2-carboxyacetyl)oxy]-4-(trimethylazaniumyl)butanoate

C10H17NO6 (247.1056)


Malonylcarnitine 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) [HMDB] Malonylcarnitine is a metabolite that accumulates with specific disruption of fatty-acid oxidation caused by the 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).