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Last updated: 2016/11/27

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BCKDHA (Human) Recombinant Protein (P01)

  • Catalog # : H00000593-P01
  • Visit Frequency :
  • Countries :
  • Specification
  • Product Description:
  • Human BCKDHA full-length ORF ( AAH07878.1, 1 a.a. - 445 a.a.) recombinant protein with GST-tag at N-terminal.
  • Sequence:
  • MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYGNISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
  • Host:
  • Wheat Germ (in vitro)
  • Theoretical MW (kDa):
  • 74.69
  • Purification:
  • Glutathione Sepharose 4 Fast Flow
  • Quality Control Testing:
  • 12.5% SDS-PAGE Stained with Coomassie Blue.

    QC Testing of H00000593-P01
  • Storage Buffer:
  • 50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.
  • Storage Instruction:
  • Store at -80°C. Aliquot to avoid repeated freezing and thawing.
  • Note:
  • Best use within three months from the date of receipt of this protein.
  • Applications
  • Enzyme-linked Immunoabsorbent Assay
  • Western Blot (Recombinant protein)
  • Antibody Production
  • Protein Array
  • Application Image
  • Enzyme-linked Immunoabsorbent Assay
  • Western Blot (Recombinant protein)
  • Antibody Production
  • Protein Array
  • 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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