HMG-CoA synthase

protein
Name=3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble)
caption=


width=
HGNCid=5007
Symbol=HMGCS1
AltSymbols=HMGCS
EntrezGene=3157
OMIM=142940
RefSeq=NM_002130
UniProt=Q01581
PDB=
ECnumber=2.3.3.10
Chromosome=5
Arm=p
Band=14
LocusSupplementaryData=-p13protein
Name=3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)
caption=


width=
HGNCid=5008
Symbol=HMGCS2
AltSymbols=
EntrezGene=3158
OMIM=600234
RefSeq=NM_005518
UniProt=P54868
PDB=
ECnumber=
Chromosome=1
Arm=p
Band=13
LocusSupplementaryData=-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).

Forms

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.

Cytosolic

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

Mitochondrial

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

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References