The BCKDHA Polyclonal Antibody from MyBioSource.com is a Rabbit Polyclonal antibody to BCKDHA, and branched chain keto acid dehydrogenase E1 subunit alpha. This antibody recognizes Human, Mouse, and Rat antigen. The BCKDHA Polyclonal Antibody has been shown to work in the following applications: Immunohistochemistry, and Western Blot.
Description
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene