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

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).

线粒体戊二酰辅酶A脱氢酶（GCDH）催化戊二酰辅酶A与FAD的反应，生成丁烯二酰辅酶A、FADH2和二氧化碳。活性酶由缺乏44个残基氨基末端线粒体靶向序列的GCDH多肽组成的四聚体。GCDH中的突变可在体内导致戊二酸尿症1型（Fu等，2004；Keyser等，2008）。