HMG-CoA synthase

Hydroxymethylglutaryl -coenzyme A synthase (short: HMG -CoA synthase ) are called enzymes that transfer an acetyl group to acetoacetyl -CoA. This reaction is part of several metabolic pathways in eukaryotes and some bacteria. In vertebrates there are two paralogous isoforms of the enzyme: the cytosolic ( HMGCS1 ) and the mitochondrial HMG -CoA synthase ( HMGCS2 ). The mitochondrial enzyme is localized in human liver and kidney and is solely responsible for the biosynthesis of ketone bodies. The cytosolic form is part of cholesterol biosynthesis in plants part of the mevalonate pathway for the synthesis of isoprenoids. Mutations in HMGCS2 gene can lead to rare inherited enzyme deficiency with severe hypoglycemia.

Catalyzed reaction

H2O CoA -SH

Acetoacetyl -CoA is acetylated to HMG-CoA.