{"product_id":"elk-es14463-npc1-rabbit-pab","title":"NPC1 rabbit pAb","description":"\u003cp\u003eNPC1 rabbit pAb is an ELK Biotechnology antibody for research workflows involving NPC1.\u003c\/p\u003e\u003ch3\u003eBackground\u003c\/h3\u003e\u003cp\u003eThis 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\u003ch3\u003eAdditional antibody information\u003c\/h3\u003e\u003ctable\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003cth\u003eSpecificity\u003c\/th\u003e\n\u003ctd\u003eThis antibody detects endogenous levels of NPC1 at Human\/Mouse\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eValidation evidence captions\u003c\/th\u003e\n\u003ctd\u003eWestern blot analysis of lysates from KB cells, primary antibody was diluted at 1:1000, 4°over night\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e","brand":"ELK Biotechnology","offers":[{"title":"Unconjugated \/ 100µL","offer_id":56185469993300,"sku":"ABM104996","price":220.41,"currency_code":"GBP","in_stock":true},{"title":"Unconjugated \/ 50µL","offer_id":56185470026068,"sku":"ABM95132","price":131.54,"currency_code":"GBP","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/1040\/1782\/2036\/files\/ES14463-c-1.jpg?v=1782035462","url":"https:\/\/www.abmium.com\/products\/elk-es14463-npc1-rabbit-pab","provider":"ABMIUM","version":"1.0","type":"link"}