glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2

Stable Identifier
R-HSA-71046
Type
Reaction
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
18775954 Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH)

Muschol, N, Mühlhausen, C, Keyser, B, Braulke, T, Ullrich, K, Christensen, E, Dickmanns, A

Hum Mol Genet 2008
15274622 Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions

Paschke, R, Wang, M, Kim, JJ, Rao, KS, Frerman, FE, Fu, Z

Biochemistry 2004
Participants
Participates
Catalyst Activity

glutaryl-CoA dehydrogenase activity of GCDH tetramer [mitochondrial matrix]

Orthologous Events
Cross References
Rhea
Authored
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