Product Information
Cite ID#EN5517873
TypePolyclonal Antibodies
HostRabbit
Positive controlMouse liver, Mouse kidney, Mouse heart, Rat kidney, Rat liver
IsotypeIgG
SummaryThe 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.
FormLiquid
Storage BufferBuffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.