beta-hydroxy-beta-methylglutaryl- lyase

β-·hy·drox·y·-·β-·meth·yl·glu·tar·yl·-· ly·ase

an enzyme, found primarily in liver and rumen epithelium that catalyzes the formation of acetyl-CoA and acetoacetate from β-hydroxy-β-methylglutaryl-CoA; a key step in ketogenesis; a deficiency of this enzyme leads to episodes of severe metabolic acidosis without ketosis.