Q03426

Mevalonate kinase (MVK) is an early enzyme in isoprenoid and sterol synthesis. Mevalonate kinase catalyzes the ATP-dependent phosphorylation of mevalonic acid to form mevalonate 5-phosphate. Mevalonate is a key intermediate, and mevalonate kinase a key early enzyme, in isoprenoid and sterol synthesis. Deficiency in MVK activity contributes to mevalonic aciduria and hyperimmunoglobulinemia D/periodic fever syndrome (HIDS). Mevalonic acid accumulates because of failure of conversion to 5-phosphomevalonic acid, which is catalyzed by mevalonate kinase. Mevalonic acid is synthesized from 3-hydroxy-3-methylglutaryl-CoA, a reaction catalyzed by HMG-CoA reductase.