3-methylglutaconyl-CoA hydratase


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3-meth·yl·glu·ta·con·yl-CoA hy·dra·tase

(meth'il-glū'tă-kon'il hī'dră-tās),
An enzyme that catalyzes the reaction of trans-3-methylglutaconyl-CoA and water to form 3-hydroxy-3-methylglutaconyl-CoA; this enzyme participates in the pathway for l-leucine degradation; a deficiency of this enzyme will result in 3-methylglutaconic aciduria.
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3-Methylglutaconyl-CoA Hydratase Deficiency(*)--Fewer than 50 cases of this disorder have been identified.
beta]-ketothiolase deficiency 3-Methylcrotonylglycine 3-Methylcrotonyl-CoA carboxylase deficiency; multiple carboxylase deficiency; HMG-CoA lyase deficiency 3-Methylglutaconate (37-42) 3-Methylglutaconyl-CoA hydratase deficiency (methylglutaconic aciduria type I); HMG-CoA lyase deficiency; 3-methylglutaconic aciduria (other than type I); respiratory chain defects (e.

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