methylglutaconyl-CoA hydratase

methylglutaconyl-CoA hydratase
meth·yl·glu·ta·con·yl-CoA hy·dra·tase (meth″əl-gloo″tə-konґəl ko-aґ hiґdrə-tās) [EC 4.2.1.18] an enzyme of the lyase class that catalyzes the hydration of 3-methylglutaconyl CoA to form 3-hydroxy-3-methylglutaryl CoA, a step in the catabolism of leucine. Deficiency of the enzyme, an autosomal recessive trait, causes 3-methylglutaconicaciduria.

Medical dictionary. 2011.

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