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Last updated: 2017/8/13

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BCKDHA 293T Cell Transient Overexpression Lysate(Denatured)

  • Catalog # : H00000593-T01
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  • Specification
  • Transfected Cell Line:
  • 293T
  • Plasmid:
  • pCMV-BCKDHA full-length
  • Host:
  • Human
  • Theoretical MW (kDa):
  • 49.06
  • Quality Control Testing:
  • Transient overexpression cell lysate was tested with Anti-BCKDHA antibody (H00000593-B01) by Western Blots.
    SDS-PAGE Gel
    QC Testing of H00000593-T01
    BCKDHA transfected lysate.
    Western Blot
    QC Testing of H00000593-T01
    Lane 1: BCKDHA transfected lysate ( 49.06 KDa)
    Lane 2: Non-transfected lysate.
  • Storage Buffer:
  • 1X Sample Buffer (50 mM Tris-HCl, 2% SDS, 10% glycerol, 300 mM 2-mercaptoethanol, 0.01% Bromophenol blue)
  • Storage Instruction:
  • Store at -80°C. Aliquot to avoid repeated freezing and thawing.
  • Applications
  • Western Blot
  • Application Image
  • Western Blot
  • Gene Information
  • Entrez GeneID:
  • 593
  • Gene Name:
  • BCKDHA
  • Gene Alias:
  • BCKDE1A,FLJ45695,MSU,MSUD1,OVD1A
  • 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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