Immunofluorescence analysis of Hela cells using NPC1 antibody (green). Blue: DAPI fluorescent DNA dye. Red: Actin filaments have been labeled with Omnimabs® 594-Phalloidin.
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.[provided by RefSeq, Aug 2009]
Immunofluorescence analysis of Hela cells using NPC1 antibody (green). Blue: DAPI fluorescent DNA dye. Red: Actin filaments have been labeled with Omnimabs® 594-Phalloidin.
Application Notes
IF:1:50-1:200
Additional Information
Form
Liquid
Storage Instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C long term. Avoid freeze / thaw cycle.