BCKDHA polyclonal, anti-human, mouse, rat

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.

Synonyms: BCKDE1A, MSU, MSUD1, OVD1A

Cellular Location: Mitochondrion matrix

Immunogen:
Recombinant protein of human BCKDHA

Gene Symbol: BCKDHA

Gene ID: 593

Swiss prot: P12694

Calculated MW: 50kDa

Recommended dilution:
WB 1:500-2000

Purity:
Affinity purification

Storage Buffer:
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:
Store at -20℃. Avoid freeze / thaw cycles.