BCKDHA antibody

Catalog No. ABIN6572016

Product Details anti-BCKDHA Antibody

Target: BCKDHA (Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This BCKDHA antibody is un-conjugated

Application: Western Blotting (WB)

Purification: Affinity purification

Immunogen: Recombinant protein of human BCKDHA

Isotype: IgG

Application Details

Application Notes: WB 1:500 - 1:2000

Restrictions: For Research Use only

Handling

Concentration: 1 mg/mL

Buffer: Buffer: PBS with 0.02 % sodium azide, 50 % glycerol,  pH 7.3.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Avoid freeze / thaw cycles.

Target Details for BCKDHA

Target: BCKDHA (Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA))

Alternative Name: BCKDHA (BCKDHA Products)

Background:

Synonyms: Branched chain alpha keto acid dehydrogenase E1 component alpha polypeptide, FLJ45695, OVD1A,2 oxoisovalerate dehydrogenase (lipoamide),2 oxoisovalerate dehydrogenase subunit alpha, mitochondrial ,2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial,BCKDE1A,BCKDH E1 alpha,BCKDH E1-alpha,BCKDHA,Branched chain alpha keto acid dehydrogenase E1 component alpha chain,Branched chain keto acid dehydrogenase E1 alpha polypeptide,Branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease),Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain,MSU,MSUD1,ODBA

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.

Molecular Weight:

Observed_MW: 50kDa

Calculated_MW: 50kDa

Gene ID: 593

UniProt: P12694