Date published: 2025-9-6

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NPC1 抗体 (G-1): sc-271335

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说明书
  • NPC1 抗体 G-1 是小鼠单克隆 IgA (kappa轻链),NPC1抗体,规格为200 µg/ml
  • 免疫human物种的NPC1的氨基酸516-630
  • 推荐用于 mouse, rat 和 human 来源的NPC1 WB, IP, IF 和 ELISA检测
  • 关于如何获取NPC1 (G-1): sc-271335免费10 µg小样,联系我们技术服务部门 (或者您当地的代理商)了解详情。
  • m-IgGκ BP-HRP是NPC1 Antibody (G-1) 适用于 WB 应用。 的首选辅助检测试剂。该试剂现可与NPC1 Antibody (G-1) 搭配使用(请参阅下面的订购信息)。有关其它 m-IgGκ BP 结合物,请参阅我们完整的小鼠 IgG 结合蛋白列表。

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関連項目

NPC1抗体(G-1)是一种IgA κ小鼠单克隆NPC1抗体(也称为NPC1抗体),可通过WB、IP、IF和ELISA检测小鼠、大鼠和人源的NPC1蛋白。NPC1抗体(G-1)为非偶联抗NPC1抗体。细胞通过两种不同的途径获得胆固醇,即在内质网中的内源性合成和通过低密度脂蛋白(LDL)受体途径的外源性摄取。NPC1是一种存在于晚期核内体和溶酶体中的蛋白质,参与胆固醇的细胞内转运。人类NPC1基因定位于18q11.2染色体上,产生经N-糖基化的蛋白质,在脑和肝中表达。NPC1含有一个富含半胱氨酸的结构域,这对蛋白质的正常功能至关重要,但高度突变。NPC1的突变导致C型尼曼-皮克病(NPC),这是一种常染色体隐性遗传病,其特征是胆固醇在核内体/溶酶体系统中非酯化积聚。胆固醇的积累会导致进行性神经退化和死亡。90%以上的NPC病例是由NPC1突变引起的,NPC患者出现多种神经系统症状,如肝脾肿大、共济失调、肌张力障碍和痴呆。

仅限研究使用。不适用于诊断和治疗用途。

Alexa Fluor® 是Molecular Probes Inc., OR., USA的商标

LI-COR®和 Odyssey® 是LI-COR Biosciences的注册商标

NPC1 抗体 (G-1) 参考文献:

  1. 亮氨酸拉链图案和固醇感应结构域的突变会使尼曼-皮克病 C1 糖蛋白失活。  |  Watari, H., et al. 1999. J Biol Chem. 274: 21861-6. PMID: 10419504
  2. NPC1 的突变突显了 NPC1 特异的富半胱氨酸保守结构域。  |  Greer, WL., et al. 1999. Am J Hum Genet. 65: 1252-60. PMID: 10521290
  3. 优雅线虫的 C 型尼曼镰刀菌病模型  |  Sym, M., et al. 2000. Curr Biol. 10: 527-30. PMID: 10801441
  4. Niemann-Pick C1 蛋白的结构和功能。  |  Ioannou, YA. 2000. Mol Genet Metab. 71: 175-81. PMID: 11001808
  5. 内源性合成的胆固醇在尼曼镰刀型 C1 细胞中的去向  |  Cruz, JC. and Chang, TY. 2000. J Biol Chem. 275: 41309-16. PMID: 11013265
  6. 尼曼-皮克病 C 型的神经元会积累神经节苷脂和未酯化胆固醇,并发生树突和轴突改变。  |  Zervas, M., et al. 2001. J Neuropathol Exp Neurol. 60: 49-64. PMID: 11202175
  7. 通过改变鞘脂运输检测 Niemann-Pick C 变体以及 NPC1 特定结构域内突变的相关性。  |  Sun, X., et al. 2001. Am J Hum Genet. 68: 1361-72. PMID: 11349231
  8. 从 C 型尼曼-皮克病(spm/spm)小鼠模型中建立永生化许旺细胞并确定其特征。  |  Watabe, K., et al. 2001. J Peripher Nerv Syst. 6: 85-94. PMID: 11446388
  9. Reovirus 感染受 NPC1 和内体胆固醇平衡的调控。  |  Ortega-Gonzalez, P., et al. 2022. PLoS Pathog. 18: e1010322. PMID: 35263388
  10. NPC1蛋白贩运的物种特异性差异影响着尼曼-皮克C型疾病的治疗反应。  |  Schultz, ML., et al. 2022. JCI Insight. 7: PMID: 36301667
  11. 依赖于 NPC1 的 KV2.1-CaV1.2 纳米域的改变推动了尼曼-皮克 C 型疾病模型中神经元的死亡。  |  Casas, M., et al. 2023. Nat Commun. 14: 4553. PMID: 37507375
  12. EXTL3和NPC1是哺乳动物感染秋沙虫多核多角体病毒的宿主因子。  |  Huang, Y., et al. 2024. Nat Commun. 15: 7711. PMID: 39231976

订购信息

产品名称产品编号规格价格数量收藏夹

NPC1 抗体 (G-1)

sc-271335
200 µg/ml
$316.00

NPC1 (G-1): m-IgGκ BP-HRP 套装

sc-551088
200 µg Ab; 40 µg BP
$354.00

Hi, is NPC1 (G-1) have been checked in Huh7 cells and CHO-K1 cells?

Asked by: Anonymous
Thank you for your question. It would be helpful if you could call us directly at 021-60936350, allowing for a more interactive discussion of this and other related questions.
Answered by: Technical Service SH
Date published: 2022-01-18

Has this antibody been validated?

Asked by: Lucy10
Thank you for your question. This antibody has been validated in Western blotting (WB) and immunofluorescent (IF) staining research applications. Our website is updated with the most current data available for our products. Please contact our Technical Service Department for further support.
Answered by: Technical Support
Date published: 2019-06-21

What is the recommended fixation method for immunofluorescence staining with NPC1 (G-1): sc-271335 monoclonal antibody?

Asked by: Germaine
Thank you for your inquiry. We recommend fixing cells for 5 minutes in -10°C methanol and allowing to air dry. The full protocol can be viewed here: https://www.scbt.com/scbt/resources/protocols/immunofluorescence-cell-staining
Answered by: Technical Support
Date published: 2017-02-24
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Rated 1 out of 5 by from No signal in WBNo detectable signals in several trials on different ovarian cancer cell extracts (dilution 1:500, O.N. incubation)
Date published: 2021-02-10
Rated 5 out of 5 by from Superb immunofluorescence cytoplasmic stainingSuperb immunofluorescence cytoplasmic staining in methanol-fixed HeLa cells. -SCBT QC
Date published: 2015-05-12
Rated 5 out of 5 by from Satisfactory Western Blot data of NPC1 expressionSatisfactory Western Blot data of NPC1 expression in ES-2 whole cell lysate. -SCBT QC
Date published: 2014-02-09
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NPC1 抗体 (G-1) is rated 3.7 out of 5 by 3.
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