Anti-Niemann Pick C1抗体(ab116038)

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
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to a region within internal sequence amino acids 590-620 of Human Niemann Pick C1

  • 阳性对照
    • NCI-H460 cell line lysates

应用

Our Abpromise guarantee covers the use of ab116038 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
WB 1/100 - 1/500. 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

Anti-Niemann Pick C1 antibody 图像

  • Anti-Niemann Pick C1 antibody (ab116038) at 1/100 dilution + NCI-H460 cell line lysates at 35 µg

    Predicted band size : 142 kDa

Anti-Niemann Pick C1 antibody (ab116038)参考文献

ab116038 has not yet been referenced specifically in any publications.

Product Wall

Thank you for your inquiry.

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...

Read More

Thank you for contacting us.

The product ab36983 was created from an immunogen at the C terminal regionf Human Niemann Pick C1 so that would not be appropriate for your needs. Of our other products, only ab116038 was crerated from and immuno...

Read More

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"