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BCKDHA Pre-design Chimera RNAi

  • Catalog # : H00000593-R01
  • Visit Frequency :
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  • Specification
  • Product Description:
  • Homo sapiens branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) (BCKDHA), mRNA.
  • Reactivity:
  • Human
  • Supplied Product:
  • DEPC water
  • Target Refseq:
  • NM_000709
  • Storage Instruction:
  • Store at -20°C, do not exceed 4 - 5 freeze-thaw cycles to ensure product integrity.
  • Note:
  • Position of the Chimera RNAi.

  • Publication Reference
  • Applications
  • RNAi Knockdown
  • Application Image
  • RNAi Knockdown
  • 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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