BCKDHA polyclonal antibody

• 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.

• 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) .

• Modification:

  Unmodified

• Molecular Weight:

  50kDa

• Storage Conditions:

  Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

• Notes:

  For research use only, not for use in diagnostic procedure.

• Specificity:

  BCKDHA polyclonal antibody detects endogenous levels of BCKDHA protein.

• Applications Notes:

  Western blot analysis of various lysates, using BCKDHA pAb at 1:2000 dilution.

• Host or Source:

  Rabbit

• CAS Number:

  9007-83-4