acyl-Coenzyme A dehydrogenase, long chain

Function

• acyl-Coenzyme A dehydrogenase
• mitochondrial fatty acid beta-oxidation system, first step
• preference for fatty acids longer than C12

acyl-CoA + acceptor <--> 2,3-dehydroacyl-CoA + reduced acceptor

Cofactor: FAD

Structure

• homotetramer
• belongs to the acyl-CoA dehydrogenase family

Compartment

mitochondrial matrix

Pathology

• defects in ACADL are the cause of long-chain acyl-CoA dehydrogenase deficiency

Notes

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

More general terms

• acyl CoA dehydrogenase

Database

• UniProt: http://www.uniprot.org/uniprot/P28330.html
• OMIM: https://mirror.omim.org/entry/201460
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=33