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HMG-CoA synthase: Wikis


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3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble)
Symbol HMGCS1
Alt. symbols HMGCS
Entrez 3157
HUGO 5007
OMIM 142940
RefSeq NM_002130
UniProt Q01581
Other data
EC number
Locus Chr. 5 p14-p13
3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)
Symbol HMGCS2
Entrez 3158
HUGO 5008
OMIM 600234
RefSeq NM_005518
UniProt P54868
Other data
Locus Chr. 1 p13-p12

HMG-CoA synthase is an enzyme which catalyzes the reaction where Acetyl-CoA condenses with acetoacetyl-CoA to form 3-hydroxy-3-methyl glutaryl-CoA (HMG-CoA). This reaction occurs at a faster rate in patients with Diabetes Insipidus type I if left untreated.



There are two different forms of the enzyme; in humans the cytosolic form has only 60.6% amino acid identity with the mitochondrial form of the enzyme.

HMG-CoA synthase reaction


The cytosolic enzymes has a key role in the production of cholesterol and other isoprenoids:


The mitochondrial form is used for the production of ketone bodies:


The gene for the mitochondrial form of the enzyme has three sterol regulatory elements in the 5' flanking region [1]. These elements are responsible for decreased transcription of the message responsible for enzyme synthesis when dietary cholesterol is high in animals: the same is observed for 3-hydroxy-3-methylglutaryl-CoA and the low density lipoprotein receptor.

External links


  1. ^ Goldstein J.L., Brown M.S. (1990) Regulation of the mevalonate pathway. Nature 343, 425-430


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