3-hydroxyisobutyryl-CoA hydrolase

3-hydroxyisobutyryl-CoA hydrolase
3-hy·droxy·iso·bu·ty·ryl-CoA hy·dro·lase (hi-drok″se-i″so-buґtə-rəl ko-aґ hiґdro-lās) [EC 3.1.2.4] an enzyme of the hydrolase class that catalyzes the cleavage of the coenzyme A (CoA) moiety from 3-hydroxyisobutyryl CoA as a step in the use of valine as a fuel. Deficiency results in toxic tissue accumulation of the CoA thioester of methacrylic acid and its cysteine compound conjugates.

Medical dictionary. 2011.

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