HMG-CoA synthase

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3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble)
Identifiers
SymbolHMGCS1
Alt. symbolsHMGCS
Entrez3157
HUGO5007
OMIM142940
RefSeqNM_002130
UniProtQ01581
Other data
EC number2.3.3.10
LocusChr. 5 p14-p13
3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)
Identifiers
SymbolHMGCS2
Entrez3158
HUGO5008
OMIM600234
RefSeqNM_005518
UniProtP54868
Other data
LocusChr. 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-methylglutaryl-CoA (HMG-CoA). There are two different forms of the enzyme; in humans the cytosolic form has only 60.6% amino acid identity with the mitochondrial from 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:

Regulation

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

References

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



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