Reaction: glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2
- in pathway: Lysine catabolism
Mitochondrial glutaryl-CoA dehydrogenase (GCDH) catalyzes the reaction of glutaryl-CoA and FAD to form crotonyl-CoA, FADH2, and CO2. The active enzyme is a tetramer of GCDH polypeptides lacking a 44-residue aminoterminal mitochondrial targeting sequence. Mutations in GCDH cause glutaric aciduria type 1 in vivo (Fu et al. 2004; Keyser et al. 2008).
Reaction - small molecule participants:
crotonyl-CoA [mitochondrial matrix]
FADH2 [mitochondrial matrix]
CO2 [mitochondrial matrix]
FAD [mitochondrial matrix]
GL-CoA [mitochondrial matrix]
Reactome.org reaction link: R-HSA-71046
======
Reaction input - small molecules:
FAD(3-)
glutaryl-CoA(5-)
Reaction output - small molecules:
crotonoyl-CoA(4-)
FADH2(2-)
carbon dioxide
Reactome.org link: R-HSA-71046