| 产品详情 |
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| Antigenic Specificity | Niemann Pick C1/NPC1 |
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| Clone | polyclonal |
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| Host Species | Rabbit |
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| Reactive Species | human, mouse, rat |
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| Isotype | n/a |
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| Format | unconjugated |
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| Size | 100ug |
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| Concentration | 0.5-1mg/ml, actual concentration varies by lot. |
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| Applications | ELISA, 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 | Rabbit IgG polyclonal Picoband™ antibody for Niemann Pick C1 detection. Tested with WB, Direct ELISA in Human;Mouse;Rat. No cross reactivity with other proteins. Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). 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 | E. coli-derived human Niemann Pick C1 recombinant protein (Position: A1022-F1278). |
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| Other Names | Niemann-Pick C1 protein; NPC1 |
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| Gene, Accession # | NPC1, UniProt: O15118 |
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| Catalog # | A00428-2 |
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| Price | |
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| Order / More Info | Niemann Pick C1/NPC1 Antibody from BOSTER BIO |
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| Product Specific References | n/a |
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