Mitochondrial fatty acid oxidation disorders

Rinaldo P, Matern D, Bennett MJ. Fatty acid oxidation disorders. Annu Rev Physiol. 2002;64():477–502. doi: 10.1146/annurev.physiol.64.082201.154705. PMID: 11826276.

Metabolites

Carnitine

Formula: C7H16NO3 (162.1130126)

CAS ID: 406-76-8

CoA

Formula: C21H36N7O16P3S (767.1152046000001)

CAS ID: 85-61-0



Enzyme

EC Number name full name note
2.3.1.-
1.1.1.211 long-chain-3-hydroxyacyl-CoA dehydrogenase long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
1.3.8.1 short-chain acyl-CoA dehydrogenase short-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.7 medium-chain acyl-CoA dehydrogenase medium-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.8 long-chain acyl-CoA dehydrogenase long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.9 very-long-chain acyl-CoA dehydrogenase very-long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
2.3.1.155 acetyl-CoA C-myristoyltransferase myristoyl-CoA:acetyl-CoA C-myristoyltransferase
2.3.1.16 acetyl-CoA C-acyltransferase acyl-CoA:acetyl-CoA C-acyltransferase
4.2.1.17 enoyl-CoA hydratase (3S)-3-hydroxyacyl-CoA hydro-lyase


Proteins

Protein ID name full name