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Tbio
HMGCS2
Hydroxymethylglutaryl-CoA synthase, mitochondrial

Protein Summary
Description
This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000369406
  • ENSP00000358414
  • ENSG00000134240
  • ENST00000544913
  • ENSP00000439495
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
disease perturbation
0.87
protein domain
0.85
kinase perturbation
0.71
disease
0.69
gene perturbation
0.67


Protein Classes
No PANTHER Classes or DTO Classes found
IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 100.06   (req: < 5)
Gene RIFs: 16   (req: <= 3)
Antibodies: 180   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 100.06   (req: >= 5)
Gene RIFs: 16   (req: > 3)
Antibodies: 180   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 6
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
Protein Sequence and Structure
Residue Counts
Protein Sequence
ProtVista Viewer
Expression Data (0 Tissues)
No expression data found
Related Tools
Target Illumination GWAS Analytics (TIGA)
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TIGA scores and ranks GWAS discovered associations according to the quantity and quality of the evidence supporting the association.
GENEVA
Thumbnail image for GENEVA
GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
Thumbnail image for GlyGen
GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
ARCHS4
Thumbnail image for ARCHS4
ARCHS4 provides access to gene-function predictions based on RNA-seq co-expression, and gene expression levels across cell and tissues.
Approved Drugs (0)
No approved drugs found
Active Ligands (0)
No active ligands found
Protein-Protein Interactions (157)
NFATC1
Tchem
Family:  TF
Novelty:  0.00130337
p_int:  0.999951148
p_ni:  0.000048852
Score:  0.309
Data Source:  BioPlex,STRINGDB
SSR3
Tbio
Novelty:  0.05838475
p_int:  0.916191786
p_ni:  0.08377421
p_wrong:  0.000034004
Data Source:  BioPlex
PDE4DIP
Tbio
Novelty:  0.03414104
p_int:  0.798004576
p_ni:  0.201974548
p_wrong:  0.000020876
Score:  0.172
Data Source:  BioPlex,STRINGDB
HMGCR
Tclin
Family:  Enzyme
Novelty:  0.00046789
Score:  0.987
Data Source:  STRINGDB
ACAT1
Tchem
Family:  Enzyme
Novelty:  0.00908928
Score:  0.982
Data Source:  STRINGDB
ACAT2
Tchem
Family:  Enzyme
Novelty:  0.01464168
Score:  0.98
Data Source:  STRINGDB
PPARA
Tclin
Family:  NR
Novelty:  0.00017403
Score:  0.973
Data Source:  STRINGDB
HADHB
Tbio
Family:  Enzyme
Novelty:  0.00969194
Score:  0.973
Data Source:  STRINGDB
HMGCL
Tbio
Family:  Enzyme
Novelty:  0.02741994
Score:  0.972
Data Source:  STRINGDB
ACAA1
Tbio
Family:  Enzyme
Novelty:  0.00710465
Score:  0.972
Data Source:  STRINGDB
Pathways (26)
Ketone body metabolism (R-HSA-74182)

Click on a row in the table to change the structure displayed.

Find Similar Targets
Items per page:
1 – 5 of 6
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Ketone body metabolism
Reactome
Metabolism
Reactome
Metabolism of lipids
Reactome
PPARA activates gene expression
Reactome
Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha)
Name
Explore in Pharos
Explore in Source
Ketone body metabolism
Metabolism
Metabolism of lipids
PPARA activates gene expression
Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha)
Viral Interactions (0)
No viral interactions found
Disease Associations ()
No disease associations found
GWAS Traits (19)
GWAS Trait
EFO ID
Study Count
SNP Count
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
blood metabolite measurement
2
2
2
89.1
2
2
0
1.1
81
red blood cell density measurement
1
1
1
79.5
hematocrit
2
2
2
78.7
amino acid measurement
1
1
1
73.7
GWAS Trait
EFO ID
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
blood metabolite measurement
2
89.1
0
1.1
81
red blood cell density measurement
1
79.5
hematocrit
2
78.7
amino acid measurement
1
73.7
Find similar targets by:
IDG Resources
No IDG generated resources found
Orthologs (12)
1 – 5 of 12
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
3-hydroxy-3-methylglutaryl-CoA synthase 2
VGNC:6865
457169
Macaque
3-hydroxy-3-methylglutaryl-CoA synthase 2
713541
Mouse
MGI:101939
15360
Rat
RGD:2804
24450
Dog
3-hydroxy-3-methylglutaryl-CoA synthase 2
VGNC:41715
607923
Species
Name
OMA
EggNOG
Inparanoid
Chimp
3-hydroxy-3-methylglutaryl-CoA synthase 2
Macaque
3-hydroxy-3-methylglutaryl-CoA synthase 2
Mouse
Rat
Dog
3-hydroxy-3-methylglutaryl-CoA synthase 2
Publication Statistics
PubMed Score 100.06
PubMed score by year
PubTator Score 69.08
PubTator score by year
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Related Publications
Items per page:
0 of 0
PMID
Year
Title