验证数据展示
经过测试的应用
| Positive WB detected in | unboiled HEK-293 cells, HEK-293 cells, HepG2 cells, unboiled mouse brain tissue, HeLa cells | 
| Positive IHC detected in | human liver cancer tissue, human placenta tissue,  human brain tissue Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0 | 
| Positive IF/ICC detected in | Neuro-2a cells, HepG2 cells | 
| Positive FC (Intra) detected in | HepG2 cells | 
推荐稀释比
| 应用 | 推荐稀释比 | 
|---|---|
| Western Blot (WB) | WB : 1:500-1:2000 | 
| Immunohistochemistry (IHC) | IHC : 1:50-1:500 | 
| Immunofluorescence (IF)/ICC | IF/ICC : 1:200-1:800 | 
| Flow Cytometry (FC) (INTRA) | FC (INTRA) : 0.40 ug per 10^6 cells in a 100 µl suspension | 
| 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. | |
产品信息
13926-1-AP targets NPC1 in WB, IHC, IF/ICC, FC (Intra), ELISA applications and shows reactivity with human, mouse samples.
| 经测试应用 | WB, IHC, IF/ICC, FC (Intra), ELISA Application Description | 
| 文献引用应用 | WB, IHC | 
| 经测试反应性 | human, mouse | 
| 文献引用反应性 | human, mouse, pig, monkey | 
| 免疫原 | CatNo: Ag4946 Product name: Recombinant human NPC1 protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 23-266 aa of BC063302 Sequence: QSCVWYGECGIAYGDKRYNCEYSGPPKPLPKDGYDLVQELCPGFFFGNVSLCCDVRQLQTLKDNLQLPLQFLSRCPSCFYNLLNLFCELTCSPRQSQFLNVTATEDYVDPVTNQTKTNVKELQYYVGQGFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPKPQPPPPPAPWTILGLD种属同源性预测 | 
| 宿主/亚型 | Rabbit / IgG | 
| 抗体类别 | Polyclonal | 
| 产品类型 | Antibody | 
| 全称 | Niemann-Pick disease, type C1 | 
| 别名 | Niemann Pick C1 protein, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC intracellular cholesterol transporter 1 | 
| 计算分子量 | 142 kDa | 
| 观测分子量 | 160-200 kDa | 
| GenBank蛋白编号 | BC063302 | 
| 基因名称 | NPC1 | 
| Gene ID (NCBI) | 4864 | 
| RRID | AB_2152050 | 
| 偶联类型 | Unconjugated | 
| 形式 | Liquid | 
| 纯化方式 | Antigen affinity purification | 
| UNIPROT ID | O15118 | 
| 储存缓冲液 | PBS with 0.02% sodium azide and 50% glycerol, pH 7.3. | 
| 储存条件 | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. | 
背景介绍
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.
实验方案
| Product Specific Protocols | |
|---|---|
| IF protocol for NPC1 antibody 13926-1-AP | Download protocol | 
| IHC protocol for NPC1 antibody 13926-1-AP | Download protocol | 
| WB protocol for NPC1 antibody 13926-1-AP | Download protocol | 
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols | 
发表文章
| Species | Application | Title | 
|---|---|---|
| Nat Commun A genome-wide CRISPR screen identifies host factors that regulate SARS-CoV-2 entry. | ||
| EMBO J Insufficiency of ciliary cholesterol in hereditary Zellweger syndrome.
 | ||
| Proc Natl Acad Sci U S A Cholesterol trafficking is required for mTOR activation in endothelial cells.
 | ||
| Proc Natl Acad Sci U S A Inhibition of sphingolipid synthesis improves outcomes and survival in GARP mutant wobbler mice, a model of motor neuron degeneration. | ||
| Cell Rep The GARP Complex Is Involved in Intracellular Cholesterol Transport via Targeting NPC2 to Lysosomes. | 













