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Last updated: 2016/10/16

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BCKDHA rabbit monoclonal antibody

  • Catalog # : H00000593-K
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  • Specification
  • Product Description:
  • Rabbit monoclonal antibody raised against a human BCKDHA peptide using ARM Technology.
  • Immunogen:
  • A synthetic peptide of human BCKDHA is used for rabbit immunization.
    Customer or Abnova will decide on the preferred peptide sequence.
  • Host:
  • Rabbit
  • Reactivity:
  • Human
  • Purification:
  • Protein A
  • Isotype:
  • IgG
  • Quality Control Testing:
  • Antibody reactive against human BCKDHA peptide by ELISA and mammalian transfected lysate by Western Blot.
  • Storage Buffer:
  • In 1x PBS, pH 7.4
  • Storage Instruction:
  • Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
  • Note:
  • 1. Customer may provide cell or tissue lysate for antibody screening.
    2. Rabbit monoclonal antibody generated by ARM technology is amenable to antibody engineering including F(ab)2, IgG, scFv and different Fc and non-Fc conjugates per customer request.
  • Library Construction:
  • Non-fusion antibody library from rabbit spleen (ARM Technology).
  • Expression:
  • Overexpression vector and transfection into 293H cell line.
  • Deliverable:
  • Up to three rabbit IgG clones of 1 mg each will be delivered to customer.
  • Applications
  • Application Image
  • Western Blot (Transfected lysate)
  • Gene Information
  • Entrez GeneID:
  • 593
  • Gene Name:
  • Gene Alias:
  • Gene Description:
  • branched chain keto acid dehydrogenase E1, alpha polypeptide
  • Gene Summary:
  • 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
  • Other Designations:
  • 2-oxoisovalerate dehydrogenase (lipoamide),branched-chain alpha-keto acid dehydrogenase complex E1 alpha subunit,maple syrup urine disease
  • Related Disease
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