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Hydroxymethylglutaryl-coenzyme A synthase (EC 184.108.40.206) (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.
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