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.
Purified antibody in PBS with 0.05% sodium azide
4°C; -20°C for long term storage
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);
For Research Use Only. Not for use in diagnostic procedures.