NPC1抗体;NPC1 Polyclonal Antibod

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 1.Liver Int. 2010 Jul;30(6):887-97. 2.Neuroscience. 2010 May 19;167(3):608-20. Specification Aliases:NPC Entrez GeneID:4864 Swissprot:O15118 clone:8D10G3 WB Predicted band size:142.2kDa Host/Isotype:Mouse IgG1 Antibody Type:Primary antibody Storage:Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. Species Reactivity:Human Immunogen:Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli. Formulation:Purified antibody in PBS with 0.05% sodium azide Application IHC:1/200 - 1/1000 FCM:1/200 - 1/400 ELISA:1/10000