MitoSciences: Very long-chain acyl-CoA dehydrogenase (VLCAD)

UniProt Number:	P49748
Alternate Names:	very long-chain specific acyl-CoA dehydrogenase, ACADVL
Structure and Function:	A mitochondrial inner membrane enzyme, VLCAD is active toward esters of long-chain and very long-chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
Disease Associations:	Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation.