Hydroxymethylglutaryl coenzyme A synthase
| Hydroxymethylglutaryl coenzyme A synthase | ||
|---|---|---|
| Identifier | ||
| Gene name (s) | HMGCS1 , HMGCS2 | |
| Enzyme classification | ||
| EC, category | 2.3.3.10 , Transferase | |
| Response type | Transfer of an acetyl group | |
| Substrate | Acetyl-CoA + acetoacetyl-CoA | |
| Products | HMG-CoA + CoA | |
| Occurrence | ||
| Parent taxon | Eukaryotes | |
Hydroxymethylglutaryl-Coenzyme A synthase ( HMG-CoA synthase for short ) are enzymes that transfer an acetyl group to acetoacetyl-CoA . This response is part of several metabolic pathways in eukaryotes and some bacteria . There are two paralogous isoforms of the enzyme in vertebrates : the cytosolic ( HMGCS1 ) and the mitochondrial HMG-CoA synthase ( HMGCS2 ). The mitochondrial enzyme is located in the liver and kidneys of humans and is solely responsible for the biosynthesis of the 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 for rare inherited enzyme deficiency with severe hypoglycemia lead.
Catalyzed reaction
+ 
+ H 2 O
+ CoA-SH + H +
-3-Hydroxy-3-methylglutaryl-CoA.svg){kind=small}
Acetoacetyl-CoA is acetylated to HMG-CoA.
Individual evidence
- ↑ a b Swiss Institute of Bioinformatics (SIB): PROSITE documentation PDOC00942. HMG-CoA synthase. Retrieved August 15, 2011 .
- ↑ UniProt P54868 , UniProt Q01581
- ↑ Orphanet: 3-hydroxy 3-methylglutaryl-CoA (HMG) synthase deficiency
Web links
- reactome.org: acetoacetyl-CoA + acetyl-CoA ⇒ HMG-CoA