Product Datasheet  
NPC1 Antibody  
Catalog Number: 24900  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot:O15118
    Gene ID:4864
  • Form of Antibody：  
  • Supplied in PBS containing 0.02% sodium azide.
  • Storage：  
  • Can be stored at -20˚C, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
  • Immunogen：  
  • Raised against a 16 amino acid peptide from near the carboxy terminus of human NPC1.
  • reactivity：  
  • Hu
  • appl_detail：  
  • other_names：  
  • Niemann-Pick disease type C1, NPC
  • Purification：  
  • Affinity chromatography purified via peptide column
  • Specificity：  
  • Applications：  
  • ELISA WB IHC
  • Background：  
  • Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease.



 
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