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hydroxymethylglutaryl-CoA lyase [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.
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