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Mapping the self-association domains of ataxin-1: Identification of novel non overlapping motifs

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1 Division of Molecular Structure, MRC National Institute of Medical Research, London, Greater London, United Kingdom
2 Randall Division for Cell and Molecular Biophysics, King's College London, London, Greater London, United Kingdom
3 British Heart Foundation Centre of Research Excellence, King's College London, London, Greater London, United Kingdom
4 Department of Molecular Neuroscience, Institute of Psychiatry, King's College London, London, Greater London, United Kingdom
DOI
10.7287/peerj.preprints.259v1
Published
Accepted
Subject Areas
Biochemistry, Cell Biology
Keywords
polyglutamine diseases, confocal microscopy, spinocerebellar ataxia type, FRET, foci, misfolding diseases
Copyright
© 2014 Menon et al.
Licence
This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, reproduction and adaptation in any medium and for any purpose provided that it is properly attributed. For attribution, the original author(s), title, publication source (PeerJ PrePrints) and either DOI or URL of the article must be cited.
Cite this article
Menon R, Soong D, de Chiara C, Holt M, McCormick J, Anilkumar N, Pastore A. 2014. Mapping the self-association domains of ataxin-1: Identification of novel non overlapping motifs. PeerJ PrePrints 2:e259v1

Abstract

The neurodegenerative spinocerebellar ataxia type 1 (SCA1) is caused by aggregation and misfolding of the ataxin-1 protein. While the pathology correlates with mutations that lead to expansion of a polyglutamine tract in the protein, other regions contribute to the aggregation process as also non-expanded ataxin-1 is intrinsically aggregation-prone and forms nuclear foci in cell. Here, we have used a combined approach based on FRET analysis, confocal microscopy and in vitro techniques to map aggregation-prone regions other than polyglutamine and to establish the importance of dimerization in self-association/foci formation. Identification of aggregation-prone regions other than polyglutamine could greatly help the development of SCA1 treatment more specific than that based on targeting the low complexity polyglutamine region.

Additional Information

Competing Interests

Annalise Pastore is an Academic Editor for PeerJ.

Author Contributions

Rajesh Menon conceived and designed the experiments, performed the experiments, analyzed the data, wrote the paper, prepared figures and/or tables, reviewed drafts of the paper.

Daniel Soong conceived and designed the experiments, performed the experiments, analyzed the data, prepared figures and/or tables, reviewed drafts of the paper.

Cesira de Chiara conceived and designed the experiments, performed the experiments, analyzed the data, wrote the paper, prepared figures and/or tables, reviewed drafts of the paper.

Mark Holt conceived and designed the experiments, performed the experiments, analyzed the data, reviewed drafts of the paper.

John McCormick performed the experiments, analyzed the data, contributed reagents/materials/analysis tools, reviewed drafts of the paper.

N. Anilkumar conceived and designed the experiments, performed the experiments, analyzed the data, reviewed drafts of the paper.

Annalisa Pastore wrote the paper, reviewed drafts of the paper.

Grant Disclosures

The following grant information was disclosed by the authors:

The work was supported by the MRC (Grant ref. U117584256), from Ataxia UK.

Funding

The work was supported by the MRC (Grant ref. U117584256), from Ataxia UK and from the British Heart Foundation. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

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