Anti-Ataxin 3抗体(ab96316)

概述

  • 产品名称Anti-Ataxin 3抗体
    参阅全部 Ataxin 3 一抗
  • 描述
    兔多克隆抗体to Ataxin 3
  • 经测试应用适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Cow, Pig
  • 免疫原

    Recombinant fragment, corresponding to a region within the N terminal amino acids 1-215 of Human Ataxin 3 (AAH33711).

  • 阳性对照
    • NT2D1, IMR32 and U-87MG cells

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab96316 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 1/500 - 1/3000. Predicted molecular weight: 42 kDa.
ICC/IF Use at an assay dependent concentration.

靶标

  • 功能Interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription. Acts as a histone-binding protein that regulates transcription. Acts as a deubiquitinating enzyme.
  • 组织特异性Ubiquitous.
  • 疾病相关Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) [MIM:109150]; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
  • 序列相似性Contains 1 Josephin domain.
    Contains 3 UIM (ubiquitin-interacting motif) repeats.
  • 细胞定位Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AT3 antibody
    • Ataxin 3 antibody
    • ataxin 3 variant h antibody
    • ataxin 3 variant m antibody
    • ataxin 3 variant ref antibody
    • Ataxin-3 antibody
    • ATX3 antibody
    • ATX3_HUMAN antibody
    • ATXN3 antibody
    • EC 3.4.22. antibody
    • JOS antibody
    • Josephin antibody
    • Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) antibody
    • Machado Joseph disease antibody
    • Machado Joseph disease protein 1 antibody
    • Machado-Joseph disease protein 1 antibody
    • Machado-Joseph disease protein 1 homolog antibody
    • MJD antibody
    • MJD gene antibody
    • MJD1 antibody
    • Olivopontocerebellar ataxia 3 antibody
    • OTTHUMP00000221583 antibody
    • OTTHUMP00000221585 antibody
    • OTTHUMP00000221586 antibody
    • OTTHUMP00000221587 antibody
    • OTTHUMP00000231995 antibody
    • OTTHUMP00000231997 antibody
    • Rsca3 antibody
    • SCA3 antibody
    • SCA3 gene antibody
    • Spinocerebellar ataxia type 3 protein antibody
    see all

Anti-Ataxin 3 antibody 图像

  • Anti-Ataxin 3 antibody (ab96316) at 1/1000 dilution + U-87MG whole cell lysate at 30 µg

    Predicted band size : 42 kDa

Anti-Ataxin 3 antibody (ab96316)参考文献

ab96316 has not yet been referenced specifically in any publications.

Product Wall

Application Immunocytochemistry/ Immunofluorescence
Blocking step Serum as blocking agent for 30 minute(s) · Concentration: 10% · Temperature: 25°C
Sample Human Cell (iPS cells generated from ATXN3 fibroblasts)
Specification iPS cells generated from ATXN3 fibroblasts
Permeabilization Yes - 0.5% triton
Fixative Paraformaldehyde
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提交于 May 30 2014

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