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

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

Stable Identifier

R-HSA-71046

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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

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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