Rabbit anti-BCKDHA Polyclonal Antibody

BCKDHA抗体;BCKDE1A,MSU,MSUD1,OVD1A

Rabbit

Human;Mouse;Rat

WB 1:500 - 1:2000

Detects a band of approximately 50kD (Predicted molecular weight: 50kD)
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1).

NCI-H460,LO2,DU145,NIH/3T3,Mouse liver,Mouse kidney,Mouse heart,Rat kidney,Rat liver

Affinity purification

Polyclonal Antibody

IgG

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

Store at -20℃. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

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.

Mitochondrion matrix