Proteintech, 86233-5-RR, NPC1 Recombinant monoclonal antibody

Size: 20ul / 100ul The NPC1 (86233-5-RR) by Proteintech is a Recombinant antibody targeting NPC1 in WB, ELISA applications with reactivity to human samples 86233-5-RR targets NPC1 in WB, ELISA applications and shows reactivity with human samples. Tested Applications Positive WB detected in: 37°C incubated HEK-293 cells Recommended dilution Western Blot (WB): WB : 1:2000-1:10000 Background Information Niemann-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. Specification Tested Reactivity: human Host / Isotype: Rabbit / IgG Class: Recombinant Type: Antibody Immunogen: 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 Full Name: Niemann-Pick disease, type C1 Calculated Molecular Weight: 142 kDa Observed Molecular Weight: 160-200 kDa GenBank Accession Number: BC063302 Gene Symbol: NPC1 Gene ID (NCBI): 4864 Conjugate: Unconjugated Form: Liquid Purification Method: Protein A purification UNIPROT ID: O15118 Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3. Storage 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.