CST, 33422T, NPC1 (E7S4N) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying NPC1. Validated for Western Blotting,Immunohistochemistry (Paraffin),Immunofluorescence (Immunocytochemistry). Available in 2 sizes. Highly specific and rigorously validated in-house, NPC1 (E7S4N) Rabbit Monoclonal Antibody (CST #33422) is ready to ship. Product Usage Information Western Blotting: 1:1000 Immunohistochemistry (Paraffin): 1:50 - 1:200 Immunofluorescence (Immunocytochemistry): 1:800 - 1:1600 Storage Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody. Protocol Available protocols: Western Blotting, Immunohistochemistry (Paraffin), Immunofluorescence (Immunocytochemistry) Specificity / Sensitivity NPC1 (E7S4N) Rabbit Monoclonal Antibody recognizes endogenous levels of total NPC1 protein. Species Reactivity: Human, Mouse, Rat Source / Purification Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Arg1266 of human NPC1 protein. Background Niemann-Pick type C (NPC) disease is a lysosomal storage disease in which endocytosed cholesterol becomes sequestered in late endosomes/lysosomes (LEs/Ls) due to mutations in either the or gene, which encode NPC1 (Niemann-Pick type C1) or NPC2 (Niemann-Pick type C2) protein (1,2). NPC1 is a multipass transmembrane protein located at the LEs/Ls membrane, and NPC2 is located in the lumen of the vesicles. NPC2 directly binds to sequestered unesterified cholesterol in the lumen and delivers the cholesterol to the N-terminal domain of membrane-bound NPC1. The cholesterol further interacts with the sterol-sensing domain of NPC1 and is eventually exported from LEs/Ls to Golgi, ER, or plasma membrane (3,4). In cells with dysfunctional NPC1, cholesterol is sequestered in the lysosome, resulting in lysosomal dysfunction and NPC. Loss of function of NPC1 leads to progressive neurodegeneration (5), neuroinflammation and myelin defects, severe neonatal liver disease and lung failure, and immune dysfunction (1,6,7). Elevated NPC1 is linked to cancer progression and drug resistance. Reagents that target NPC1 activity provide opportunities for disease treatment strategies (8,9). Alternate Names FLJ98532; Niemann-Pick C1 protein; Niemann-Pick disease, type C1; NPC; NPC intracellular cholesterol transporter 1; NPC1; POGZ; SLC65A1; truncated Niemann-Pick C1 Specification REACTIVITY: H M R SENSITIVITY: Endogenous MW (kDa): 190 Source/Isotype: Rabbit IgG