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).
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
enoyl-CoA hydratase activity of ECHS1 hexamer [mitochondrial matrix]
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