very long-chain specific acyl-CoA dehydrogenase, mitochondrial; VLCAD (ACADVL, VLCAD)

Function

• mitochondrial fatty acid beta-oxidation system, 1st step
• active toward esters of long-chain & very-long chain fatty acids, i.e. palmitoyl-CoA & stearoyl-CoA ,

Cofactor: FAD

Structure

• homodimer
• belongs to the acyl-CoA dehydrogenase family

Compartment

mitochondrial inner membrane

Alternative splicing

named isoforms=2

Pathology

• defects in ACADVL are the cause of VLCAD deficiency

Notes

• a number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues

More general terms

• acyl CoA dehydrogenase
• mitochondrial membrane protein

Additional terms

• very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=37
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:37; -.
• OMIM: https://mirror.omim.org/entry/201475
• OMIM: https://mirror.omim.org/entry/609575
• UniProt: http://www.uniprot.org/uniprot/P49748.html