Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

hydroxymethylglutaryl-CoA synthase

Name from literature

HMG CoA synthase

Pathway from literature

mevalonate pathway/ketogenesis

Pathway from KEGG

Amino Acid Metabolism; Valine, leucine and isoleucine degradation; map00280

Lipid Metabolism; Synthesis and degradation of ketone bodies; map00072

Carbohydrate Metabolism; Butanoate metabolism; map00650


Mouse (10090)

Genome localization

3 F2.2|3 48.0 cM[15360 ], -[208715 ],

Rate-limiting Description

"This SREBP activation covers not only rate-limiting enzymes such as HMG CoA synthase and reductase that have been well established as SREBP targets, but also all the enzyme genes in the cholesterol synthetic pathway tested here." (11485325)

"The mode of action of these compounds in the 50-100 microM concentration range appeared to be by increasing lipid excretion from the body and by inhibiting rate-limiting enzyme activities for the de novo synthesis of lipids and cholesterol (e.g., phosphatidylate phosphohydrolase, ATP-dependent citrate lyase, cytoplasmic acetyl coenzyme A ." (CoA synthetase, HMG CoA reductase, and acetyl CoA carboxylase))

Regulatory Information

Upstream transcription factor


Regulatory type










transcriptional factor;PPARalpha(19013)

"The PPARalpha targeted genes CPT-II and HMG-CoA synthase are potential regulators of mitochondrial fatty acid oxidation and ketogenesis in hTNFalpha transgenic mice, and the increased propionyl-CoA level found is a possible inhibitor of these processes. " (15893958#10869548)

transcriptional factor;PPAR(19013)

"The mRNA profiles of BMAL1 and of the PPAR target genes acyl-CoA oxidase (AOX), 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) synthase and cellular retinol binding protein II (CRBPII) were measured in intestine. " (16020918)

Gene ontology

Gene ontology

GO:0008299 (P) isoprenoid biosynthetic process [P54869, Q8JZK9 ];
GO:0005737 (C) cytoplasm [Q8JZK9 ];
GO:0005743 (C) mitochondrial inner membrane [P54869 ];
GO:0004421 (F) hydroxymethylglutaryl-CoA synthase activity [P54869, Q8JZK9 ];
GO:0006695 (P) cholesterol biosynthetic process [P54869, Q8JZK9 ];

Subcellular localization


mitochondrion [P54869 ];

cytoplasm [Q8JZK9 ];



P54869; Q8JZK9

Entrez Gene

15360; 208715

  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min