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ß-hydroxy-ß-methylglutaryl- lyase


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

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.