Hmgcs2 Gene Summary [Rat]
Enables hydroxymethylglutaryl-CoA synthase activity. Involved in several processes, including cellular response to insulin stimulus; cellular response to lipopolysaccharide; and ketone body biosynthetic process. Located in mitochondrial matrix. Biomarker of extrahepatic cholestasis. Human ortholog(s) of this gene implicated in HMG-CoA synthase 2 deficiency. Orthologous to human HMGCS2 (3-hydroxy-3-methylglutaryl-CoA synthase 2). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Hmgcs2 |
| Official Name | 3-hydroxy-3-methylglutaryl-CoA synthase 2 [Source:RGD Symbol;Acc:2804] |
| Ensembl ID | ENSRNOG00000019120 |
| Bio databases IDs | |
| Aliases | 3-hydroxy-3-methylglutaryl-CoA synthase 2 |
| Synonyms | 1300002P16, 3-hydroxy-3-methylglutaryl-CoA synthase 2, 3-HYDROXY-3-METHYLGLUTARYL-COENZYME A SYNTHASE, 3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2, HMCS2, HMG COA SYNTHASE, Hmg Coa Synthetase, Hmgcs1, mHS, Mt3h3mg, Mthmg-coa Synthase |
| Species | Rat, Rattus norvegicus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in rat Hmgcs2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- cond_enzymes
- 3-hydroxy-3-methylglutaryl CoA synthase, prokaryotic clade
- 3-hydroxy-3-methylglutaryl-CoA-synthase, eukaryotic clade
- hydroxymethylglutaryl-CoA synthase
- enzyme
- Hydroxymethylglutaryl-coenzyme A synthase C terminal
- identical protein binding
Pathways
Biological processes and signaling networks where the Hmgcs2 gene in rat plays a role, providing insight into its function and relevance in health or disease.
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- non-insulin-dependent diabetes mellitus
- deficiency of hydroxymethylglutaryl-coa synthase
- epithelial cancer
- lung squamous cell carcinoma
- squamous cell lung cancer
- mitochondrial HMG-CoA synthase deficiency
- liver cancer
- organismal death
- atopic dermatitis
- hepatocellular carcinoma
role in cell
- expression in
- apoptosis
- proliferation
- ketogenesis in
- beta-oxidation in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Mitochondria
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the rat Hmgcs2 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- farnesyl diphosphate biosynthetic process, mevalonate pathway
- midgut development
- acetyl-CoA metabolic process
- response to starvation
- response to cAMP
- ketone body biosynthetic process
- response to xenobiotic stimulus
- cellular response to insulin stimulus
- cellular response to amino acid stimulus
- response to ethanol
- cellular response to fatty acid
- kidney development
- liver development
- multicellular organismal response to stress
- response to metal ion
- response to linoleic acid
- response to testosterone stimulus
- lung development
- response to monosaccharide stimulus
- response to temperature stimulus
- cholesterol biosynthetic process
- response to growth hormone stimulus
- response to nutrient
- adipose tissue development
- cellular response to lipopolysaccharide
- cellular response to glucocorticoid stimulus
- response to glucagon stimulus
- response to triglyceride
- response to prostaglandin F stimulus
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- identical protein binding
- hydroxymethylglutaryl-CoA synthase activity