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Last updated: 2017/12/10

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BCKDHB FISH Probe

  • Catalog # : FA0171
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  • Specification
  • Product Description:
  • Made to order FISH probes for identification of gene amplification using Fluorescent In Situ Hybridization Technique. (Technology)
  • Supplied Product:
  • DAPI Counterstain (1500 ng/mL ) 250 uL
  • Storage Instruction:
  • Store at 4°C in the dark.
  • Origin:
  • Human
  • Source:
  • Genomic DNA
  • Notice:
  • We strongly recommend the customer to use FFPE FISH PreTreatment Kit 1 (Catalog #: KA2375 or KA2691) for the pretreatment of Formalin-Fixed Paraffin-Embedded (FFPE) tissue sections.
  • Regulation Status:
  • For research use only (RUO)
  • Applications
  • Application Image
  • Fluorescent In Situ Hybridization (Cell)
  • Gene Information
  • Entrez GeneID:
  • 594
  • Gene Name:
  • BCKDHB
  • Gene Alias:
  • E1B,FLJ17880,dJ279A18.1
  • Gene Description:
  • branched chain keto acid dehydrogenase E1, beta polypeptide
  • Gene Summary:
  • Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq
  • Other Designations:
  • 2-oxoisovalerate dehydrogenase beta subunit,E1b-beta subunit of the branched-chain complex,OTTHUMP00000018048,OTTHUMP00000018049,branched chain alpha-ketoacid dehydrogenase E1-beta subunit,branched chain keto acid dehydrogenase E1 beta polypeptide,branche
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