Mouse Monoclonal Antibody to NPC1

Mouse IgG1

MW: 142.2kDa
Enterez GeneID: 4864
General Information: 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. ;
Immunogen: Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli.
Size and Concentration: 100ug/1mg/ml
Supplied As: Lyophilized Powder from 100ul of Purified antibody in PBS with 0.05% sodium azide
Reconstitution and Storage: Reconstituted with 100ul sterile DI H2O, at stored at 4C or -20C for short or long term storage.
Applications: ELISA: 1 to 10000; WB: 1 to 500 - 1 to 2000; IHC: 1 to 200 - 1 to 1000
References: 1. Am J Pathol. 2012 Aug; 181(2):487-98. ; 2. J Korean Med Sci. 2011 May; 26(5):647-53. ;