Validation Data Gallery
Tested Applications
| Positive WB detected in | 37°C incubated HEK-293 cells |
| Positive IF/ICC detected in | HepG2 cells |
Recommended dilution
| Application | Dilution |
|---|---|
| Western Blot (WB) | WB : 1:2000-1:10000 |
| Immunofluorescence (IF)/ICC | IF/ICC : 1:200-1:800 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
Product Information
86233-5-RR targets NPC1 in WB, IF/ICC, ELISA applications and shows reactivity with human samples.
| 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 相同性解析による交差性が予測される生物種 |
| 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 | |
| Form | Liquid |
| Purification Method | Protein A purification |
| UNIPROT ID | O15118 |
| Storage Buffer | PBS with 0.02% sodium azide and 50% glycerol{{ptg:BufferTemp}}7.3 |
| Storage Conditions | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
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.
Protocols
| Product Specific Protocols | |
|---|---|
| IF protocol for NPC1 antibody 86233-5-RR | Download protocol |
| WB protocol for NPC1 antibody 86233-5-RR | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |