{"product_id":"npc1-rabbit-pab-es14463","title":"NPC1 rabbit pAb - ES14463","description":"\u003cp\u003eNPC1 rabbit pAb\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: ES14463-50, ES14463-100\u003c\/p\u003e\n\n\u003cp\u003eCitations, Manuals and MSDS Available upon request.\u003c\/p\u003e\n\n\u003cp\u003eBackground: 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.[provided by RefSeq, Aug 2009],\u003c\/p\u003e \n\n\u003cp\u003eSource: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB\u003c\/p\u003e\n\n\u003cp\u003eDilution: WB 1: 500-2000\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human; Mouse\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Synthesized peptide derived from human NPC1 AA range: 1073-1123\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: -20°C\/1 year\u003c\/p\u003e\n\n\u003cp\u003eClonality: Polyclonal\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003eConcentration: 1 mg\/ml\u003c\/p\u003e\n\n\u003cp\u003eHuman Gene ID: 4864\u003c\/p\u003e\n\n\u003cp\u003eHuman SWISS Prot NO: O15118\u003c\/p\u003e\n\n\u003cp\u003eSubcellular Location: Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ELK Bio","offers":[{"title":"50μL","offer_id":45201628430515,"sku":"ES14463-50","price":207.2,"currency_code":"CAD","in_stock":true},{"title":"100μL","offer_id":45201628463283,"sku":"ES14463-100","price":347.2,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/download_360x_cd43f9ef-e071-4e47-bb9e-82d9c9d8714a.png?v=1767108058","url":"https:\/\/afsbio.com\/products\/npc1-rabbit-pab-es14463","provider":"AFSBio Inc.","version":"1.0","type":"link"}