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| Antigenic Specificity | Niemann-Pick Disease, Type C1 (NPC1) (aa 34-174) |
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| Clone | 8D10G3 |
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| Host Species | Mouse |
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| Reactive Species | human |
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| Isotype | IgG1 |
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| Format | purified |
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| Size | 0.1 mg |
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| Concentration | n/a |
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| Applications | ELISA,Flow Cytometry (FACS),Immunohistochemistry (IHC),Western Blotting (WB) |
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| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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| 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. |
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| Immunogen | Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli. |
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| Other Names | im:7149020|wu:fb53a12|wu:fc29a12|A430089E03Rik|C85354|D18Ertd139e|D18Ertd723e|lcsd|nmf164|spm|NPC|Cdig2 |
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| Gene, Accession # | Gene ID: 4864 |
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| Catalog # | ABIN1724840 |
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| Price | |
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| Order / More Info | Niemann-Pick Disease, Type C1 (NPC1) (aa 34-174) Antibody from ANTIBODIES-ONLINE GmbH |
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| Product Specific References | PubMed: 20408952, 20167265 |
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