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.,HMGCS2, Hydroxymethylglutaryl-CoA synthase, mitochondrial, 3-hydroxy-3-methylglutaryl coenzyme A synthase, 3-hydroxy-3-methylglutaryl-CoA synthase 2,Cancer,Signal Transduction,Endocrine & Metabolism,Mitochondrial metabolism,Mitochondrial markers,Lipid Metabolism,Cholesterol Metabolism,Cardiovascular,Lipids,HMGCS2