Anti-Niemann Pick C1抗体(ab106534)

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
  • 特异性Will not cross-react with NPC2
  • 经测试应用适用于: ICC/IF, WB, IHC-Pmore 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
    • Human kidney lysate, Mouse kidney tissue.

应用

Our Abpromise guarantee covers the use of ab106534 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
ICC/IF Use a concentration of 20 µg/ml.
WB Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 142 kDa.
IHC-P Use a concentration of 2.5 µg/ml. Perform heat mediated antigen retrieval via the microwave method before commencing with IHC staining protocol.

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

Anti-Niemann Pick C1 antibody 图像

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

    Predicted band size : 142 kDa
  • ab106534, at 2.5 ug/ml, staining Niemann Pick C1 in Mouse kidney tissue by immunohistochemistry.
  • Immunofluorescence of NPC1 in Mouse Kidney cells using ab106534 at 20 ug/ml.

Anti-Niemann Pick C1 antibody (ab106534)参考文献

ab106534 has not yet been referenced specifically in any publications.

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Unfortunately, I don't think that any of the http://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.unip...

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