BCKDHA Polyclonal Antibody

Catalog Number:

31741

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P12694
NCBI Gene ID:593

Form of Antibody:

Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4.

Storage/Stability:

Immunogen:

Recombinant fusion protein of human BCKDHA (NP_000700.1).

Purification:

Affinity purification

Specificity/Sensitivity:

Applications:

WB

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.

References:

appl_detail:

WB 1:500 - 1:2000

Datasheet PDF