Overview

  • Product name
  • Description
    Rabbit polyclonal to Ataxin 3
  • Tested applications
    Suitable for: WB, ICC/IFmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Pig
  • Immunogen

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

  • Positive control
    • NT2D1, IMR32 and U-87MG cells

Properties

Applications

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.

Application Abreviews Notes
WB 1/500 - 1/3000. Predicted molecular weight: 42 kDa.
ICC/IF Use at an assay dependent concentration.

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

  • 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. 

References

ab96316 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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
Username

Abcam user community

Verified customer

Submitted 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

Abcam user community

Verified customer

Submitted 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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