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Human Molecular Genetics Advance Access originally published online on August 22, 2005
Human Molecular Genetics 2005 14(19):2893-2909; doi:10.1093/hmg/ddi321
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© The Author 2005. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Ataxin-2 and huntingtin interact with endophilin-A complexes to function in plastin-associated pathways

Markus Ralser1,{dagger}, Ute Nonhoff1,{dagger}, Mario Albrecht2, Thomas Lengauer2, Erich E. Wanker3, Hans Lehrach1 and Sylvia Krobitsch1,*

1Max Planck Institute for Molecular Genetics, Ihnestrasse 73, 14195 Berlin, Germany, 2Max Planck Institute for Informatics, Stuhlsatzenhausweg 85, 66123 Saarbrücken, Germany and 3Max Delbrueck Center for Molecular Medicine, Robert-Roessle-Strasse 10, 13092 Berlin, Germany

* To whom correspondence should be addressed. Tel: +49 3084131351; Fax: +49 3084131380; Email: krobitsc{at}molgen.mpg.de

Received July 6, 2005; Revised August 10, 2005; Accepted August 18, 2005

Spinocerebellar ataxia type 2 is an inherited neurodegenerative disorder that is caused by an expanded trinucleotide repeat in the SCA2 gene, encoding a polyglutamine stretch in the gene product ataxin-2. Although evidence has been provided that ataxin-2 is involved in RNA metabolism, the physiological function of ataxin-2 remains unclear. Here, we demonstrate that ataxin-2 interacts with two members of the endophilin family, endophilin-A1 and endophilin-A3. To elucidate the physiological implications of these interactions, we exploited yeast as a model system and discovered that expression of ataxin-2 as well as both endophilin proteins is toxic for yeast lacking the SAC6 gene product fimbrin, a protein involved in actin filament organization and endocytotic processes. Intriguingly, expression of huntingtin, another polyglutamine protein interacting with endophilin-A3, was also toxic in {Delta}sac6 yeast. These effects can be suppressed by simultaneous expression of one of the two human fimbrin orthologs, L- or T-plastin. Moreover, we have discovered that ataxin-2 associates with L- and T-plastin and that overexpression of ataxin-2 leads to accumulation of T-plastin in mammalian cells. Thus, our findings suggest an interplay between ataxin-2, endophilin proteins and huntingtin in plastin-associated cellular pathways.

{dagger}The authors wish to be known that, in their opinion, the first two authors should be regarded as joint First Authors.


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