beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA

Mitochondrial ethylglutaconyl-CoA hydratase (AUH) catalyzes the hydrolysis of beta-methylglutaconyl-CoA to yield beta-hydroxy-beta-methylglutaryl-CoA (IJlst et al. 2002; Narisawa et al. 1986). Crystallographic studies have shown the active enzyme to be a hexamer of AUH polypeptides whose amino-terminal 67 residues, a mitochondrial targeting sequence, have been removed ((Kurimoto et al. 2001).

线粒体乙基乙内酰基辅酶A水合酶（AUH）催化β-甲基乙内酰基辅酶A的水解反应，生成β-羟基-β-甲基戊二酰辅酶A（IJlst 等人，2002年；Narisawa 等人，1986年）。晶体学研究表明，活性酶由AUH多肽组成的六聚体，其氨基末端67个残基，即线粒体靶向序列已被去除（Kurimoto 等人，2001年）。