製品の概要

  • 製品名
  • 製品の詳細
    Rabbit polyclonal to Ataxin 3
  • アプリケーション
    適用あり: WB, ICC/IFmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: 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).

  • ポジティブ・コントロール
    • NT2D1, IMR32 and U-87MG cells

法規制情報

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB 1/500 - 1/3000. Predicted molecular weight: 42 kDa.
ICC/IF Use at an assay dependent concentration.

ターゲット情報

  • 機能
    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.
  • 組織特異性
    Ubiquitous.
  • 関連疾患
    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.
  • 配列類似性
    Contains 1 Josephin domain.
    Contains 3 UIM (ubiquitin-interacting motif) repeats.
  • 細胞内局在
    Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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

画像

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

    Predicted band size : 42 kDa
    10% SDS PAGE
  • ab96316 (1:250) staining Ataxin 3 in human brain SK-N-SH cells by Immunocytochemistry / Immunoflorescence. Cells were fixed in 4% paraformaldehyde at RT for 15 mins. 

参考文献

ab96316 has not yet been referenced specifically in any publications.

レビューと Q&A

Application
Western blot
Sample
Human Cell lysate - whole cell (HeLa)
Gel Running Conditions
Reduced Denaturing
Loading amount
20 µg
Specification
HeLa
Blocking step
Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
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投稿 Mar 31 2017

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
Username

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Verified customer

投稿 May 30 2014

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

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