Gene: [05p1/HMGCS1] 3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble);

GEN

The characteristic feature of the HMGCS and HMGCR (3-hydroxy-3- methylglutaryl coenzyme A reductase) genes is the 5'-untranslated region complex structure containing multiple transcription initiation sites and an intron with a variable donor splice site."

FUN

[1] The HMGCS enzyme is involved in mevalonic acid metabolism as well as in biosynthesis of cholesterol and ubiquinone progenitors.
[2] Systematic name: 3-hydroxy-3-methylglutaryl coenzyme A acetoacetyl-CoA lyase. The catalyzed reaction: (S)-3-hydroxy-3-methylglutaryl-CoA + CoA = = acetyl-CoA + H(2)O + acetoacetyl-CoA."

FAG

[1] Two consecutive reactions in cholesterol biosynthesis are catalyzed by 3-hydroxy-3-methylglutaryl coenzyme A synthase and 3-hydroxy-3-methylglutaryl coenzyme A reductase, which are encoded by unlinked genes located on the different arms of chr 5 (GEM:05q1/HMGCR).
[2] On mitochondrial HMG-synthase see GEM:01p1/HMGCS2."

REF

PRO,SEQ,EXP "Gil G &: PNAS, 84, N7, 1863-1866, 1987
LOC,CYG "Leonard S &: PNAS, 83, N7, 2187-2189, 1986
LOC,MOL,EVO "Mehrabian M &: JBC, 261, 16249-16255, 1986

SWI

SWISSPROT: Q01581

KEY

lip, ster, ats

CLA

coding, basic

LOC

05 p14-13

MIM

MIM: 142940

EZN

ENZYME: 4.1.3.5

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