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MABS440 Anti-NPC2 Antibody, clone 13G4

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MABS440
100 μg  
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      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWBMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMABS440
      DescriptionAnti-NPC2 Antibody, clone 13G4
      Alternate Names
      • Epididymal secretory protein E1
      • NPC2
      • Human epididymis-specific protein 1
      • He1
      • Niemann-Pick disease type C2 protein
      Background InformationEpididymal secretory protein E1 (UniProt P61916; also known as Human epididymis-specific protein 1, He1, Niemann-Pick disease type C2 protein) is encoded by the NPC2 (also known as HE1) gene (Gene ID 10577) in human. Niemann-Pick C1 & C2 (NPC1 & NPC2) proteins are involved in the transport of cholesterol and other lipids out of late endosomes and lysosomes. NPC1 is a large membrane spanning glycoprotein mainly located in the late endosomes, while NPC2 is a small soluble protein that binds nonesterified cholesterol with high affinity. NPC1 and NPC2 defects affect the intracellular trafficking of unesterified cholesterol and other lipids, causing the rare autosomal recessive lysosomal storage disorder known as Niemann-Pick type C disease. The majority of NPC patients have a defect in NPC1 (95%), while only 5% of the NPC cases are due to mutations in NPC2.
      References
      Product Information
      FormatPurified
      PresentationPurified mouse monoclonal IgG1κ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-NPC2 Antibody, clone 13G4 is an antibody against NPC2 for use in Western Blotting.
      Key Applications
      • Western Blotting
      Application NotesWestern Blotting Analysis: A representative lot detected the intracellular organelle protein NPC2 in whole cell lysates, but not in plasma membrane extracts, from SV-589 human skin fibroblasts (Das, A., et al. (2013). Proc. Natl. Acad. Sci. U. S. A. 110(26):10580-10585).
      Biological Information
      ImmunogenGST-tagged recombinant human NPC2.
      Clone13G4
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityClone 13G4 detects Niemann-Pick disease type C2 protein (NPC2). Reactivity toward the spliced isoform 2 (UniProt P61916-2) has not been determined.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • HE1
      • NPC2
      Purification MethodProtein G Purified
      UniProt Number
      Molecular Weight~17 kDa observed. 16.57 kDa (isoform 1) and 13.42 kDa (isoform 2) calculated. A broad, smeared banding pattern in the range from ~17 to 25 kDa is typically seen.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in HT1080 human fibrosarcoma cell lysate.

      Western Blotting Analysis: 0.5 µg/mL of this antibody detected NPC2 in 10 µg of HT1080 human fibrosarcoma cell lysate.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 μg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Référence GTIN
      MABS440 04055977329513