NPC1 Primary Antibody
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.
2.Neuroscience. 2010 May 19;167(3):608-20.
![Figure 1: Western blot analysis using NPC1 mAb against human NPC1 recombinant protein. (Expected MW is 37.6 kDa)](https://cdn.shopify.com/s/files/1/0604/2661/7019/products/30501rwb.jpg?v=1666700184)
![Figure 2: Western blot analysis using NPC1 mAb against HEK293 (1) and NPC1 (AA: 34-174)-hIgGFc transfected HEK293 (2) cell lysate.](https://cdn.shopify.com/s/files/1/0604/2661/7019/products/30501twb.jpg?v=1666700184)
![Figure 3: Immunohistochemical analysis of paraffin-embedded liver cancer tissues using NPC1 mouse mAb with DAB staining.](https://cdn.shopify.com/s/files/1/0604/2661/7019/products/30501ihc-1.jpg?v=1666700184)
![Figure 4: Immunohistochemical analysis of paraffin-embedded esophageal cancer tissues using NPC1 mouse mAb with DAB staining.](https://cdn.shopify.com/s/files/1/0604/2661/7019/products/30501ihc-2.jpg?v=1666700184)
![Figure 5: Flow cytometric analysis of Hela cells using NPC1 mouse mAb (green) and negative control (red).](https://cdn.shopify.com/s/files/1/0604/2661/7019/products/30501fcm.jpg?v=1666700184)
![Black line: Control Antigen (100 ng); Purple line: Antigen(10ng); Blue line: Antigen (50 ng); Red line: Antigen (100 ng);](https://cdn.shopify.com/s/files/1/0604/2661/7019/products/30501ea.jpg?v=1666700184)