{"product_id":"nsj-rq4395-100ug","title":"NPC1 Antibody \/ Niemann Pick C1, 100 ug","description":"\u003cp\u003eNiemann-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.\u003c\/p\u003e","brand":"NSJ Bioreagents","offers":[{"title":"100 ug","offer_id":51550178509080,"sku":"RQ4395","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/get_image_e0d90a6e-d3a5-4ba5-baf1-d7d764c6e6d1.jpg?v=1768307866","url":"https:\/\/danabiosci.com\/products\/nsj-rq4395-100ug","provider":"Dana Bioscience","version":"1.0","type":"link"}