NPC1 Primary Antibody
|Formulation||Purified antibody in PBS with 0.05% sodium azide|
|Immunogen||Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli.|
|Shipping Information||This product will ship in a box containing blue ice at a temperature of 4°C. Learn More|
|IHC_P(Immunohistochemistry)||1/200 - 1/1000|
|WB (Western Blot)||1/500 - 1/2000|
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Figure 1: Western blot analysis using NPC1 mAb against human NPC1 recombinant protein. (Expected MW is 37.6 kDa)
Figure 2: Western blot analysis using NPC1 mAb against HEK293 (1) and NPC1 (AA: 34-174)-hIgGFc transfected HEK293 (2) cell lysate.
Figure 3: Immunohistochemical analysis of paraffin-embedded striated muscle tissues using NPC1 mouse mAb with DAB staining.
Figure 4: Immunohistochemical analysis of paraffin-embedded esophageal cancer tissues using NPC1 mouse mAb with DAB staining.
Black line: Control Antigen (100 ng); Purple line: Antigen(10ng); Blue line: Antigen (50 ng); Red line: Antigen (100 ng);
|Description||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. |
|References (references)||1. Liver Int. 2010 Jul;30(6):887-97. |
2. Neuroscience. 2010 May 19;167(3):608-20.