BCKDHA polyclonal antibody

Product Specifications

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.

CAS Number

9007-83-4

Structure Composition

1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Product Name Alternative

BCKDHA; BCKDE1A; MSU; MSUD1; OVD1A

Swiss Prot

P12694

Reactivity

Human, Mouse, Rat

Immunogen

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

Conjugation

Unconjugated

Applications

WB, IF/ICC

Dilution

WB,1:500 - 1:2000|IF/ICC,1:50 - 1:200

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .