glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2 Authored: D'Eustachio, P, 2003-06-24 00:00:00 EC Number: 1.3.99.7 Edited: D'Eustachio, P, 2003-06-24 00:00:00 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). Pubmed15274622 Pubmed18775954 Reactome Database ID Release 4371046 Reactome, http://www.reactome.org ReactomeREACT_1409