acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain or medium chain acyl CoA dehydrogenase

Function

• acyl-Coenzyme A dehydrogenase
• mitochondrial fatty acid beta-oxidation system, first step
• preference for C6-C12 fatty acids.
• specific for acyl chain lengths of 4-16
• mitochondrial fatty acid beta-oxidation system, 1st step

• 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 ACADM are the cause of medium-chain acyl-CoA dehydrogenase deficiency

Laboratory

• medium-chain acyl CoA dehydrogenase leukocytes
• medium-chain acyl CoA dehydrogenase fibroblasts

Notes

• a 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/P11310.html
• OMIM: https://mirror.omim.org/entry/607008
• OMIM: https://mirror.omim.org/entry/201450
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=34