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).
Fu, Z, Wang, M, Paschke, R, Rao, KS, Frerman, FE, Kim, JJ
Keyser, B, Mühlhausen, C, Dickmanns, A, Christensen, E, Muschol, N, Ullrich, K, Braulke, T
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