概述

  • 产品名称Anti-CLN3抗体
    参阅全部 CLN3 一抗
  • 描述
    小鼠单克隆抗体to CLN3
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Recombinant Fragment
    预测可用于: Human
  • 免疫原

    Recombinant full length protein, corresponding to amino acids 1-439 of Human CLN3

性能

应用

Our Abpromise guarantee covers the use of ab54687 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
  • 应用说明WB: Use at a concentration of 1-5 µg/ml.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.
    • 疾病相关Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3.
    • 序列相似性Belongs to the battenin family.
    • 翻译后修饰Highly glycosylated.
      Farnesylation is important for trafficking to lysosomes.
    • 细胞定位Lysosome membrane. Late endosome.
    • Information by UniProt
    • 数据库链接
    • 别名
      • Batten disease protein antibody
      • Battenin antibody
      • BTS antibody
      • Ceroid lipofuscinosis neuronal 3 antibody
      • Ceroid lipofuscinosis neuronal 3 juvenile (Batten Spielmeyer Vogt disease) antibody
      • Ceroid lipofuscinosis neuronal 3 juvenile antibody
      • CLN 3 antibody
      • CLN3 antibody
      • CLN3_HUMAN antibody
      • JNCL antibody
      • MGC102840 antibody
      • Protein CLN3 antibody
      see all

    Anti-CLN3 antibody 图像

    • Western blot against tagged recombinant protein immunogen using ab54687 CLN3 antibody at 1ug/ml. Predicted band size of immunogen is 74 kDa.

      This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Anti-CLN3 antibody (ab54687)参考文献

    ab54687 has not yet been referenced specifically in any publications.

    Product Wall

    Abreviews
    Abcam has not validated the combination of species/application used in this Abreview.
    Application Western blot
    Loading amount 30 µg
    Gel Running Conditions Reduced Denaturing
    Sample Human Cell lysate - whole cell (RPE-1 cells)
    Specification RPE-1 cells
    Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
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    提交于 Jun 04 2013

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