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 | NPC1 fusion protein Ag4946 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 | NPC intracellular cholesterol transporter 1, Niemann-Pick C1 protein, Niemann Pick disease, type C1, Niemann Pick C1 protein |
| 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 | |
|---|---|
| WB protocol for NPC1 antibody 13926-1-AP | Download protocol |
| IHC protocol for NPC1 antibody 13926-1-AP | Download protocol |
| IF 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. |
