Overview

  • Product name
  • Description
    Rabbit polyclonal to Ataxin 3
  • Host species
    Rabbit
  • Tested applications
    Suitable for: ICC/IF, IHC-P, WBmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Recombinant full length protein corresponding to Human Ataxin 3 aa 1-364.
    Sequence:

    MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERMRMAE GGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQ RLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLAL FLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLK EQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADLR RAIQLSMQGSSRNISQDMTQTSGTNLTSEELRKRREAYFEKQQQKQQQQQ QQQQQGDLSGQSSHPCERPATSSGALGSDLGKACSPFIMFATFTLYLTYE LHVIFALHYSSFPL


    Database link: P54252

  • Positive control
    • MCF­7 cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab175265 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF Use at an assay dependent concentration.
IHC-P 1/50 - 1/200.

ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.

WB 1/500 - 1/2000. Predicted molecular weight: 42 kDa.

Target

  • Function
    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.
  • Tissue specificity
    Ubiquitous.
  • Involvement in disease
    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.
  • Sequence similarities
    Contains 1 Josephin domain.
    Contains 3 UIM (ubiquitin-interacting motif) repeats.
  • Cellular localization
    Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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

Images

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human lung cancer tissue labelling Ataxin 3 with ab175265 at 1/200. Magnification: 400x.
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of rat kidney tissue labelling Ataxin 3 with ab175265 at 1/200. Magnification: 400x.
  • Immunocytochemistry/Immunofluorescence analysis of HeLa cells using ab175265. Blue DAPI for nuclear staining.
  • Anti-Ataxin 3 antibody (ab175265) at 1/500 dilution + MCF­7 cell lysate

    Predicted band size: 42 kDa

References

ab175265 has not yet been referenced specifically in any publications.

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

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