{"product_id":"proteintech-86233-5-rr","title":"Proteintech, 86233-5-RR, NPC1 Recombinant monoclonal antibody","description":"Size: 20ul \/ 100ul\nThe NPC1 (86233-5-RR) by Proteintech is a Recombinant antibody targeting NPC1 in WB, ELISA applications with reactivity to human samples\n86233-5-RR targets NPC1 in WB, ELISA applications and shows reactivity with human samples.\n\u003cb\u003eTested Applications\u003c\/b\u003e\nPositive WB detected in: 37°C incubated HEK-293 cells\n\u003cb\u003eRecommended dilution\u003c\/b\u003e\nWestern Blot (WB): WB : 1:2000-1:10000\n\u003cb\u003eBackground Information\u003c\/b\u003e\nNiemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by the accumulation of unesterified cholesterol and other lipids in the endolysosomal system. NPC disease results from a defect in either of two distinct cholesterol-binding proteins: a transmembrane protein, NPC1, and a small soluble protein, NPC2. NPC1 or NPC2 deficiency models showed that the functions of these two proteins within lysosomes are linked closely. NPC1 is a typical transmembrane protein and contains a number of modification sites for glycosylation. Defects in NPC1 are the cause of Niemann-Pick disease type C1 which exhibits highly variable clinical phenotype. Moreover, NPC1 may play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.\n\u003cb\u003eSpecification\u003c\/b\u003e\nTested Reactivity: human\nHost \/ Isotype: Rabbit \/ IgG\nClass: Recombinant\nType: Antibody\nImmunogen: CatNo: Ag4946 Product name: Recombinant human NPC1 protein Source: e coli. -derived, PGEX-4T Tag: GST Domain: 23-266 aa of BC063302 Sequence: QSCVWYGECGIAYGDKRYNCEYSGPPKPLPKDGYDLVQELCPGFFFGNVSLCCDVRQLQTLKDNLQLPLQFLSRCPSCFYNLLNLFCELTCSPRQSQFLNVTATEDYVDPVTNQTKTNVKELQYYVGQGFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPKPQPPPPPAPWTILGLD Predict reactive species\nFull Name: Niemann-Pick disease, type C1\nCalculated Molecular Weight: 142 kDa\nObserved Molecular Weight: 160-200 kDa\nGenBank Accession Number: BC063302\nGene Symbol: NPC1\nGene ID (NCBI): 4864\nConjugate: Unconjugated\nForm: Liquid\nPurification Method: Protein A purification\nUNIPROT ID: O15118\nStorage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3.\nStorage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20 o C storage. 20ul sizes contain 0.1% BSA.","brand":"Proteintech","offers":[{"title":"Default Title","offer_id":46888791605417,"sku":"86233-5-RR","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/proteintech-86233-5-rr","provider":"Iright","version":"1.0","type":"link"}