Tags: Protein.
Indented line3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the HMGCS2 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.