HMGCS2 (gene)

3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is a is_associated_with::protein in humans that is encoded by the HMGCS2 is_associated_with::gene.

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].