β-hydroxy-β-methylglutaryl-CoA

β-hydroxy-β-methylglutaryl-CoA
A key intermediate in the synthesis of ketone bodies, of steroids, and of farnesyl and geranyl derivatives. SYN: 3-hydroxy- 3-methylglutaryl-CoA.
- β-hydroxy- β-methylglutaryl- lyase 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.
- β-hydroxy- β-methylglutaryl- reductase an enzyme that catalyzes the rate-limiting step of cholesterol biosynthesis : β-hydroxy-β-methylglutaryl-CoA + 2NADPH + 2H+ → (R)-mevalonate + 2NADP+ + CoA.
- β-hydroxy- β-methylglutaryl- synthase an enzyme in mitochondria that catalyzes the reaction of acetyl-CoA with acetoacetyl-CoA and water to form (S)-β-hydroxy-β-methylglutaryl-CoA and coenzyme A, a step required for both ketogenesis and steroidogenesis to occur.

Medical dictionary. 2011.

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