HMGCS2 Recombinant Rabbit Monoclonal Antibody [JE54-76] (HA720083)

UNIPROT #

P54868

PROTEIN NAME

HMGCS2

SYNONYMS

3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial) antibody;3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 antibody;3 hydroxy 3 methylglutaryl coenzyme A synthase antibody;3-hydroxy-3-methylglutaryl coenzyme A synthase antibody;HMCS2_HUMAN antibody;HMG CoA synthase antibody;HMG-CoA synthase antibody;HMGCS 2 antibody;HMGCS2 antibody;Hydroxymethylglutaryl CoA synthase antibody;Hydroxymethylglutaryl CoA synthase mitochondrial antibody;Hydroxymethylglutaryl-CoA synthase antibody;mitochondrial antibody

SUBCELLULAR LOCATION

Mitochondrion.

FUNCTION

3-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 second and rate-limiting reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting, by addition of a third acetyl group to acetoacetyl-CoA, producing HMG-CoA. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.