NPC1 Mouse Monoclonal Antibody

Description

EnkiLife offers the NPC1 Mouse Monoclonal Antibody. This antibody reacts with Human species and is suitable for IHC,ELISA,FC application. Alternative Names of Immunogen: NPC. The product boasts remarkable high specificity, outstanding reproducibility, and exceptional affinity, making it a reliable and superior choice for your needs. Dilution Ratio: IHC 1:200-1:1000,ELISA 1:5000-1:20000,FC 1:200-1:400 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.