ECHS1 hydrates methacrylyl-CoA

Stable Identifier
R-HSA-70870
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
methacrylyl-CoA + H2O <=> beta-hydroxyisobutyryl-CoA
Locations in the PathwayBrowser
General
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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

Yamada, K, Aiba, K, Kitaura, Y, Kondo, Y, Nomura, N, Nakamura, Y, Fukushi, D, Murayama, K, Shimomura, Y, Pitt, J, Yamaguchi, S, Yokochi, K, Wakamatsu, N

J. Med. Genet. 2015
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
enoyl-CoA hydratase activity of ECHS1 hexamer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Reviewed
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