{"product_id":"rq4395","title":"NPC1 Antibody \/ Niemann Pick C1","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 \/ Rabbit","offer_id":46767321841888,"sku":"RQ4395","price":439.0,"currency_code":"KRW","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0601\/9330\/8896\/files\/get_image_155b3448-e3c9-47f0-b802-580b47aa7b30.jpg?v=1734468037","url":"https:\/\/rndmate.com\/products\/rq4395","provider":"알앤디메이트","version":"1.0","type":"link"}