PROSITE documentation PDOC00942Hydroxymethylglutaryl-coenzyme A synthase active site
Hydroxymethylglutaryl-coenzyme A synthase (EC 2.3.3.10) (HMG-CoA synthase) catalyzes the condensation of acetyl-CoA with acetoacetyl-CoA to produce HMG-CoA and CoA [1].
In vertebrates there are two isozymes located in different subcellular compartments: a cytosolic form which is the starting point of the mevalonate pathway which leads to cholesterol and other sterolic and isoprenoid compounds and a mitochondrial form responsible for ketone body biosynthesis. HMG-CoA is also found in other eukaryotes such as insect, plants and fungi.
A cysteine is known to act as the catalytic nucleophile in the first step of the reaction, the acetylation of the enzyme by acetyl-CoA. We have used the conserved region around this active site residue as a signature pattern.
Last update:April 2003 / Text revised.
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| 1 | Authors | Rokosz L.L. Boulton D.A. Butkiewicz E.A. Sanyal G. Cueto M.A. Lachance P.A. Hermes J.D. |
| Title | Human cytoplasmic 3-hydroxy-3-methylglutaryl coenzyme A synthase: expression, purification, and characterization of recombinant wild-type and Cys129 mutant enzymes. | |
| Source | Arch. Biochem. Biophys. 312:1-13(1994). | |
| PubMed ID | 7913309 |
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