Gene Information
Gene NameHMGCS2
OrganismHomo sapiens (Human)
Gene Length508
Protein NamesHydroxymethylglutaryl-CoA synthase, mitochondrial (HMG-CoA synthase) (EC 2.3.3.10) (3-hydroxy-3-methylglutaryl coenzyme A synthase)
Target Namehydroxymethylglutaryl-coa synthase 2
Target TypeLiterature-reported target
Gene Age332 ( ENSG00000134240 )
Evolutionary StageCellular_organisms
AlphaFoldDBP54868
Gene CardHMGCS2
Uniprot IDP54868
PfamPF08540; PF01154
In ASDno
Allosteric Predictionyes (0.496691996)
Ortholog in AnimalsAlpaca_ HMGCS2 ( ortholog_one2one )
Dog_ HMGCS2 ( ortholog_one2one )
GuineaPig_ HMGCS2 ( ortholog_one2one )
Macaque_ HMGCS2 ( ortholog_one2one )
Mouse_ Hmgcs2 ( ortholog_one2one )
Pig_ HMGCS2 ( ortholog_one2many )
Rat_ Hmgcs2 ( ortholog_one2one )
Rabbit_ HMGCS2 ( ortholog_one2one )
OhnologsHMGCS1
ParalogsHMGCS1
Tissueliver
Tissue SpecificityExpression in liver is 200-fold higher than in any other tissue. Low expression in colon, kidney, testis, and pancreas. Very low expression in heart and skeletal muscle (PubMed:7893153, PubMed:21952825, PubMed:16940161). Not detected in brain (PubMed:21952825). {ECO:0000269|PubMed:16940161, ECO:0000269|PubMed:21952825, ECO:0000269|PubMed:7893153}.;
[Isoform 3]: Highest expression detected in heart and skeletal muscle. {ECO:0000269|PubMed:21952825}.
Pharmacological Animal ModelsNA
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 2WYA  (X-ray  1.70A 
DBSNP rs28937320  rs768707273  rs775528207  rs137852640  rs746217014  rs1237226874  rs137852636  rs761373362  rs137852638  rs1002548815  rs918691885  rs752626288  rs137852639  rs758519315 
Subcellular location [CC]Mitochondrion {ECO:0000250|UniProtKB:P22791}.
Mouse PG classificationNA
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