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Anti-Niemann Pick C1抗体(ab106534)

Submit a review Q&A (1)References (1)
Western blot - Anti-Niemann Pick C1 antibody (ab106534)
  • Western blot - Anti-Niemann Pick C1 antibody (ab106534)

Key features and details

  • Rabbit polyclonal to Niemann Pick C1
  • Suitable for: ICC/IF, IHC-P, WB
  • Reacts with: Mouse, Human
  • Isotype: IgG

选择批间可重复性更高的重组抗体

Product image
Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)
  • 研究可靠 —— 各批次间结果一致且可重复
  • 长期批量供应 —— 采用重组技术，可实现快速生产
  • 首次实验即可成功 —— 经过大量验证确认了特异性
  • 符合伦理标准 —— 产品不含动物成分

概述

  • 产品名称

    Anti-Niemann Pick C1抗体
    参阅全部 Niemann Pick C1 一抗

  • 描述

    兔多克隆抗体to Niemann Pick C1

  • 宿主

    Rabbit

  • 特异性

    Will not cross-react with NPC2

  • 经测试应用

    适用于: ICC/IF, IHC-P, WBmore details

  • 种属反应性

    与反应: Mouse, Human

  • 免疫原

    Synthetic peptide, 16 aa from near the carboxy terminus of human NPC1 (NP_000262).

  • 阳性对照

    • WB: HepG2 cell lysate; Human kidney lysate.

  • 常规说明

    The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.

    If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As

应用

The Abpromise guarantee

Abpromise™承诺保证使用ab106534于以下的经测试应用

“应用说明”部分 下显示的仅为推荐的起始稀释度；实际最佳的稀释度/浓度应由使用者检定。

应用 Ab评论 说明
ICC/IF
Use at an assay dependent concentration.
IHC-P
Use at an assay dependent concentration.
WB
Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 142 kDa.
说明
ICC/IF
Use at an assay dependent concentration.
IHC-P
Use at an assay dependent concentration.
WB
Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 142 kDa.

靶标

  • 功能

    Involved in the intracellular trafficking of cholesterol. 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.

  • 疾病相关

    Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPDC1) [MIM:257220]. A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected.

  • 序列相似性

    Belongs to the patched family.
    Contains 1 SSD (sterol-sensing) domain.

  • 结构域

    A cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes.

  • 翻译后修饰

    Glycosylated.

  • 细胞定位

    Late endosome membrane. Lysosome membrane.
  • Information by UniProt

  • 数据库链接

    • 别名

      • Niemann Pick C1 protein precursor antibody
      • Niemann Pick disease, type C1 antibody
      • Niemann-Pick C1 protein antibody
      • NPC antibody
      • NPC1 antibody
      • NPC1_HUMAN antibody
      see all

    图片

    • Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Anti-Niemann Pick C1 antibody (ab106534) at 1 µg/ml + HepG2 (human liver hepatocellular carcinoma cell line) cell lysate

      Predicted band size: 142 kDa

    • Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Anti-Niemann Pick C1 antibody (ab106534) at 1 µg/ml + Human kidney tissue lysate at 15 µg

      Predicted band size: 142 kDa

    文献 (1)

    发表研究结果有使用 ab106534？请让我们知道，以便我们可以引用本数据表中的参考文章。

    ab106534 被引用在 1 文献中.

    • Erwood S  et al. Modeling Niemann-Pick disease type C in a human haploid cell line allows for patient variant characterization and clinical interpretation. Genome Res 29:2010-2019 (2019). PubMed: 31754021

    客户评价及客户问答

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    Question

    Inquiry: Good afternoon, I was wondering if the Anti-Niemann Pick C1 antibody (ab36983) could be usefull to detect the homologous protein in yeast. Indeed, I was looking for a antibody against Ncr1p (NPC1 homologous yeast protein), but it is not available. I've looked for the peptide sequence specific for your antibody, but is not available in the datasheet of this antibody. Hope you could help with this. Thank you so much

    Read More
    Answer

    Thank you for your inquiry.

    Unfortunately, I don't think that any of the https://www.abcam.com/index.html?t=158346&pt=1 we have in our catalog is suitable for the detection of the yeast Niemann-Pick type C-related protein 1 (http://www.uniprot.org/uniprot/Q12200).
    I compared the immunogen sequence of each antibodywith the yeast protein and the maximum score is only 35%.

    I would like to recommend checking the Biocompare website which has an excellent antibody search facility that includes many suppliers. The link is: http://www.biocompare.com

    I hope this information is nevertheless helpful to you. Please do not hesitate to contact me if you have any further questions in this regard.

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