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).
Muschol, N, Mühlhausen, C, Keyser, B, Braulke, T, Ullrich, K, Christensen, E, Dickmanns, A
Paschke, R, Wang, M, Kim, JJ, Rao, KS, Frerman, FE, Fu, Z
glutaryl-CoA dehydrogenase activity of GCDH tetramer [mitochondrial matrix]
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