Reaction: beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA
- in pathway: Branched-chain amino acid catabolism
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 aminoterminal 67 residues, a mitochondrial targeting sequence, have been removed ((Kurimoto et al. 2001).
Reaction - small molecule participants:
bHMG-CoA [mitochondrial matrix]
bMC-CoA [mitochondrial matrix]
H2O [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70785
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Reaction input - small molecules:
trans-3-methylglutaconyl-CoA
water
Reaction output - small molecules:
(3S)-3-hydroxy-3-methylglutaryl-CoA(5-)
Reactome.org link: R-HSA-70785