Reaction: ECHS1 hydrates methacrylyl-CoA
- in pathway: Branched-chain amino acid catabolism
Mitochondrial ECHS1 (enoyl-CoA hydratase) hexamer catalyzes the reversible addition of water to MACR-CoA (methacrylyl-CoA) to form bHIB-CoA (beta-hydroxybutyryl-CoA). Partially purified ECHS1 catalyzes this reaction with moderate efficiency in vitro, and patients deficient in the enzyme accumulate and excrete MACR-CoA (Yamada et al. 2015).
Reaction - small molecule participants:
bHIB-CoA [mitochondrial matrix]
MACR-CoA [mitochondrial matrix]
H2O [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70870
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Reaction input - small molecules:
methacrylyl-CoA
water
Reaction output - small molecules:
(S)-3-hydroxyisobutyryl-CoA
Reactome.org link: R-HSA-70870