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Last updated: 2016/12/4
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NPC1 monoclonal antibody (M02), clone 4H2

  • Catalog # : H00004864-M02
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  • Specification
  • Product Description:
  • Mouse monoclonal antibody raised against a partial recombinant NPC1.
  • Immunogen:
  • NPC1 (AAH63302, 151 a.a. ~ 250 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
  • Sequence:
  • GFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPK
  • Host:
  • Mouse
  • Reactivity:
  • Human
  • Isotype:
  • IgG2a Kappa
  • Quality Control Testing:
  • Antibody Reactive Against Recombinant Protein.

    QC Testing of H00004864-M02
    Western Blot detection against Immunogen (36.63 KDa) .
  • Storage Buffer:
  • In 1x PBS, pH 7.4
  • Storage Instruction:
  • Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
  • Publication Reference
  • Applications
  • Western Blot (Cell lysate)
  • Western Blot (Cell lysate)
  • NPC1 monoclonal antibody (M02), clone 4H2. Western Blot analysis of NPC1 expression in HeLa.
  • PDF DownloadProtocol Download
  • Sandwich ELISA (Recombinant protein)
  • Sandwich ELISA (Recombinant protein)
  • Detection limit for recombinant GST tagged NPC1 is approximately 0.03ng/ml as a capture antibody.
  • PDF DownloadProtocol Download
  • ELISA
  • Application Image
  • Western Blot (Cell lysate)
  • Western Blot (Cell lysate)
  • enlarge
  • Western Blot (Recombinant protein)
  • Sandwich ELISA (Recombinant protein)
  • Sandwich ELISA (Recombinant protein)
  • enlarge
  • ELISA
  • Gene Information
  • Entrez GeneID:
  • 4864
  • Gene Name:
  • NPC1
  • Gene Alias:
  • NPC
  • Gene Description:
  • Niemann-Pick disease, type C1
  • Gene Summary:
  • This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments
  • Other Designations:
  • -
  • Gene Pathway
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