Protein Name 3-hydroxy-3-methylglutaryl-CoA synthase 2
Organism Homo sapiens
Gene ID 3158
Gene Symbol


UniProt P54868 (HMCS2_HUMAN), A0A140VJL2 (A0A140VJL2_HUMAN)
Relationships Total Number of functionally related compound(s) : 64
Total Number of Articles : 72

3-hydroxy-3-methylglutaryl-CoA synthase 2

Gene Summary

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]

  • hydroxymethylglutaryl-CoA synthase, mitochondrial
  • 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)
  • 3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)
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