{"product_id":"niemann-pick-c1-recombinant-rabbit-mab-s-5243-s0b60149","title":"Niemann Pick C1 Recombinant Rabbit mAb (S-5243)","description":"\u003ch4\u003eProduct Specification\u003c\/h4\u003e\u003cdiv class=\"responsive-table product-detail-table details-table\"\u003e\n\u003cbr\u003e\u003ctable style=\"width: 100%; height: auto;\"\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eHost\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eRabbit\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eAntigen\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eNiemann Pick C1\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eSynonyms\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eNPC intracellular cholesterol transporter 1; Niemann-Pick C1 protein; NPC1\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eLocation\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eLysosome, Endosome\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eAccession\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eO15118\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eClone Number\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eS-5243\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eAntibody Type\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eRecombinant mAb\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eIsotype\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eIgG\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eApplication\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eWB, IHC-P, ICC\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eReactivity\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eHu, Ms, Rt, Mk\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePositive Sample\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eU-87 MG, HT-1080, SK-BR-3, PC-3, 3T3-L1, Neuro-2a, rat liver, COS-7\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePurification\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eProtein A\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eConcentration\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003e0.5 mg\/ml\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eConjugation\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eUnconjugated\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePhysical Appearance\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eLiquid\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eStorage Buffer\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003e\u003cp\u003ePBS, 40% Glycerol, 0.05% BSA, 0.02% sodium azide\u003c\/p\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eStability \u0026amp; Storage\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003e\u003cp\u003e12 months from date of receipt \/ reconstitution, -20 °C as supplied\u003c\/p\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e\n\u003c\/div\u003e\u003ch4\u003eDilution\u003c\/h4\u003e\u003cdiv class=\"responsive-table product-detail-table details-table\"\u003e\n\u003cbr\u003e\u003ctable style=\"width: 60%; height: auto;\"\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd\u003e\u003cstrong\u003eapplication\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd\u003e\u003cstrong\u003edilution\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd\u003e\u003cstrong\u003especies\u003c\/strong\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd\u003eWB\u003c\/td\u003e\n\u003ctd\u003e1:1000-1:2000\u003c\/td\u003e\n\u003ctd\u003eHu, Ms, Rt, Mk\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd\u003eIHC-P\u003c\/td\u003e\n\u003ctd\u003e1:250\u003c\/td\u003e\n\u003ctd\u003eHu, Ms, Rt\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd\u003eICC\u003c\/td\u003e\n\u003ctd\u003e1:500\u003c\/td\u003e\n\u003ctd\u003eHu, Ms\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e\n\u003c\/div\u003e\u003ch4\u003eBackground\u003c\/h4\u003e\u003cdiv\u003e\u003cp\u003e\u003cspan style=\"color:#000000\"\u003eNiemann\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e–\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003ePick C1 (NPC1) is a large (~142 \u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003ekDa\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e) polytopic integral membrane glycoprotein that resides predominantly in the limiting membranes of late endosomes and lysosomes, where it functions as a sterol-sensing shuttle: its N-terminal \u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e“\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003echolesterol-binding pocket\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e”\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e captures cholesterol (and other hydrophobic metabolites) from the luminal leaflet, a ring of 13 trans-membrane helices forms a hydrophobic conduit that is gated by cytosolic loop 6, and the C-terminal \u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e“\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003eNPC2-interacting\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e”\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e domain receives cholesterol from the soluble lysosomal protein NPC2, thereby enabling net export of cholesterol to the cytoplasmic leaflet for redistribution to other organelles; pathogenic mutations in NPC1 block this lipid efflux, causing the fatal neurovisceral disorder Niemann\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003e–\u003c\/span\u003e\u003cspan style=\"color:#000000\"\u003ePick disease type C1 characterized by lysosomal lipid accumulation, neurodegeneration and premature death.\u003c\/span\u003e\u003c\/p\u003e\u003c\/div\u003e","brand":"Starter","offers":[{"title":"25μl","offer_id":43096505450571,"sku":"S0B60149-25μl","price":100.0,"currency_code":"USD","in_stock":true},{"title":"100µl","offer_id":43096505483339,"sku":"S0B60149-100µl","price":350.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/8375\/1499\/files\/3a8b214f025a457c962ab6c6098ee556.jpg?v=1788238966","url":"https:\/\/www.antbioinc.com\/products\/niemann-pick-c1-recombinant-rabbit-mab-s-5243-s0b60149","provider":"AntBio","version":"1.0","type":"link"}