Niemann Pick C1 Rabbit mAb
Target | NPC1 |
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Product Type | Antibody |
Application | ICC, IF, IHC (P), WB |
Clonality | Monoclonal |
Isotype | IgG |
Immunogen | A synthetic peptide corresponding to a sequence within amino acids 1179-1278 of human Niemann Pick C1 (O15118). |
Host Species | Rabbit |
Species Reactivity | Human, Mouse, Rat |
Formulation | PBS with 0.02% sodium azide,0.05% BSA,50% glycerol |
Research Area | Cancer, Immunology, Metabolism |
Description/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. |
Protocols | ICC, IF, IHC (P), WB |
Molecular Weight | 142kDa |
Purity | Affinity purification |
Regulatory Statement | For Research Use Only. Not for use in diagnostic procedures. |