Product Datasheet  
Niemann Pick C1 Rabbit mAb  
Catalog Number: 77061  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • O15118
  • Form of Antibody：  
  • Storage：  
  • Store at -20℃. Avoid freeze / thaw cycles.|Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
  • Immunogen：  
  • A synthesized peptide derived from human Niemann Pick C1.
  • reactivity：  
  • Human,Mouse,Rat
  • appl_detail：  
  • WB 1:500 - 1:2000
    IHC 1:50 - 1:200
    IF 1:50 - 1:200
  • other_names：  
  • NPC1; NPC; Niemann-Pick C1 protein
  • Purification：  
  • Affinity purification
  • Specificity：  
  • Applications：  
  • WB,IHC,IF
  • Background：  
  • 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.



 
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