hydroxymethylglutaryl-CoA lyase

hydroxymethylglutaryl-CoA lyase
hy·droxy·meth·yl·glu·ta·ryl-CoA ly·ase (hi-drok″se-meth″əl-glooґtə-rəl ko-aґ liґās) [EC 4.1.3.4] an enzyme of the lyase class that catalyzes the cleavage of 3-hydroxy-3-methylglutaryl CoA to form acetyl coenzyme A and acetoacetate. The reaction is a step in ketogenesis and in the catabolism of leucine. Deficiency of the enzyme, an autosomal recessive trait, causes 3-hydroxy-3-methylglutaricaciduria.

Medical dictionary. 2011.

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