Product Description

BCKDHA Polyclonal Antibody

Sizes: 60μL, 120μL, 200μL

Catalogue Numbers: E-AB-93313-60, E-AB-93313-120, E-AB-93313-200

Citations, Manuals and MSDS Available upon request.

Abbreviation: BCKDHA

Target Synonym: BCKDHA; BCKDE1A; MSU; MSUD1; OVD1A; 2-oxoisovalerate dehydrogenase subunit alpha; mitochondrial

Conjugation: Unconjugated

Host: Rabbit

Species Reactivity: Human

Application: WB, IF

Isotype: IgG

Clonality: Polyclonal

UNIProt ID: P12694

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.

Concentration: 1mg/mL

Immunogen: Recombinant fusion protein of human BCKDHA

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

Purification Method: Affinity purification

Dilution: WB 1:500-1:2000, IF 1:50-1:200

Calculated MW: 50kDa

Storage: -20°C/One year. Avoid freeze / thaw cycles.

Shipping: Ice bag

Research Use Only