Reactome | ECHS1 hydrates methacrylyl-CoA

ECHS1 hydrates methacrylyl-CoA

Stable Identifier

R-HSA-70870

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(2E)-2-methylpropenoyl-CoA + H2O => (S)-3-hydroxyisobutanoyl-CoA, methacrylyl-CoA + H2O <=> beta-hydroxyisobutyryl-CoA

ReviewStatus

5/5

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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).

Literature References

PubMed ID	Title	Journal	Year
26251176	Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion Shimomura, Y, Yamaguchi, S, Nomura, N, Yamada, K, Kondo, Y, Pitt, J, Murayama, K, Aiba, K, Wakamatsu, N, Nakamura, Y, Fukushi, D, Yokochi, K, Kitaura, Y	J. Med. Genet.	2015