Anti-Niemann Pick C2抗体(ab77453)

概述

  • 产品名称Anti-Niemann Pick C2抗体
    参阅全部 Niemann Pick C2 一抗
  • 描述
    小鼠单克隆抗体to Niemann Pick C2
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length human Niemann Pick C2 protein (AAH02532) with a 26 kDa tag.

  • 阳性对照
    • Niemann Pick C2 transfected 293T cell lysate

性能

应用

Our Abpromise guarantee covers the use of ab77453 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 Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 17 kDa.
ELISA Use at an assay dependent dilution.

靶标

  • 功能May be involved in the regulation of the lipid composition of sperm membranes during the maturation in the epididymis.
  • 组织特异性Epididymis.
  • 疾病相关Defects in NPC2 are the cause of Niemann-Pick disease type C2 (NPDC2) [MIM:607625]. 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 C2 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.
  • 序列相似性Belongs to the NPC2 family.
  • 细胞定位Secreted.
  • Information by UniProt
  • 数据库链接
  • 别名
    • EDDM1 antibody
    • Epididymal protein 1 antibody
    • Epididymal secretory protein antibody
    • Epididymal secretory protein E1 antibody
    • hE1 antibody
    • Human epididymis-specific protein 1 antibody
    • Niemann-Pick disease type C2 antibody
    • Niemann-Pick disease type C2 protein antibody
    • NPC intracellular cholesterol transporter 2 antibody
    • NPC2 antibody
    • NPC2_HUMAN antibody
    • Tissue specific secretory protein antibody
    see all

Anti-Niemann Pick C2 antibody 图像

  • Anti-Niemann Pick C2 antibody (ab77453) at 5 µg/ml + immunogen at 0.2 µg

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/5000 dilution

    Predicted band size : 17 kDa
    Observed band size : 43 kDa (why is the actual band size different from the predicted?)
  • All lanes : Anti-Niemann Pick C2 antibody (ab77453) at 5 µg/ml

    Lane 1 : Niemann Pick C2 transfected 293T cell lysate
    Lane 2 : Non transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 17 kDa
    Observed band size : 17 kDa

Anti-Niemann Pick C2 antibody (ab77453)参考文献

ab77453 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab77453.
Please use the links above to contact us or submit feedback about this product.

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