BCKDHA Rabbit pAb
Target | BCKDHA |
---|---|
Product Type | Antibody |
Application | WB |
Clonality | Polyclonal |
Isotype | IgG |
Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
Host Species | Rabbit |
Species Reactivity | Human, Mouse, Rat |
Formulation | PBS with 0.02% sodium azide,50% glycerol |
Research Area | Cancer, Immunology |
Description/Background | 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. |
Protocols | WB |
Molecular Weight | 50kDa |
Purity | Affinity purification |
Regulatory Statement | For Research Use Only. Not for use in diagnostic procedures. |