|PROSITE documentation PDOC00942
Hydroxymethylglutaryl-coenzyme A synthase active site
Hydroxymethylglutaryl-coenzyme A synthase (EC 126.96.36.199) (HMG-CoA synthase)
catalyzes the condensation of acetyl-CoA with acetoacetyl-CoA to produce HMG-CoA and CoA .
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.
April 2003 / Text revised.
PROSITE method (with tools and information) covered by this documentation:
|HMG_COA_SYNTHASE, PS01226; Hydroxymethylglutaryl-coenzyme A synthase active site (PATTERN)
C is the active site residue
|Sequences in UniProtKB/Swiss-Prot known to belong to this class detected by PS01226:
||17 true positives with 1 false negative.
|Other sequence(s) in UniProtKB/Swiss-Prot detected by PS01226:
|Matching PDB structures:
||Rokosz L.L., Boulton D.A., Butkiewicz E.A., Sanyal G., Cueto M.A., Lachance P.A., Hermes J.D.
||Human cytoplasmic 3-hydroxy-3-methylglutaryl coenzyme A synthase: expression, purification, and characterization of recombinant wild-type and Cys129 mutant enzymes.
||Arch. Biochem. Biophys. 312:1-13(1994).
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