acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain

Function

• acyl-Coenzyme A dehydrogenase
• mitochondrial fatty acid beta-oxidation system, 1st step
• preference for C4-C6 fatty acids

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

butanoyl-CoA + acceptor <--> 2-butenoyl-CoA + reduced acceptor

Cofactor: FAD

Structure

• homotetramer
• belongs to the acyl-CoA dehydrogenase family

Compartment

mitochondrial matrix

Pathology

• defects in ACADS are the cause of short-chain acyl-CoA dehydrogenase deficiency

More general terms

• acyl CoA dehydrogenase

Database

• UniProt: http://www.uniprot.org/uniprot/P16219.html
• OMIM: https://mirror.omim.org/entry/606885
• OMIM: https://mirror.omim.org/entry/201470
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=35