Glutaryl-CoA dehydrogenase, mitochondrial (GCD, GCDH)

Function

• catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA & CO2 in the degradative pathway of L-lysine, L-hydroxylysine, & L-tryptophan metabolism
• uses electron transfer flavoprotein as its electron acceptor
• amino-acid metabolism:
  • lysine degradation
  • tryptophan metabolism
• short isoform is inactive

Glutaryl-CoA + acceptor   <-->
              crotonoyl-CoA + CO2 + reduced acceptor

Cofactor: FAD

Structure

• homotetramer
• belongs to the acyl-CoA dehydrogenase family

Compartment

mitochondrial matrix

Alternative splicing

named isoforms=2

Expression

• the 2 isoforms have been found in fibroblasts & liver
• deficiency would suggest expression in basal ganglia

Pathology

• defects in GCDH are the cause of glutaric acidemia type 1

Laboratory

• GCDH gene mutation

More general terms

• dehydrogenase
• mitochondrial protein
• flavoprotein

Database

• OMIM: https://mirror.omim.org/entry/231670
• OMIM: https://mirror.omim.org/entry/608801
• UniProt: http://www.uniprot.org/uniprot/Q92947.html