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First published online 1 September 2004
doi: 10.1242/dev.01352
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1 Division of Basic Sciences, Fred Hutchinson Cancer Research Center, Seattle,
WA 98109, USA
2 Howard Hughes Medical Institute, Seattle, WA 98109, USA
3 Department of Zoology, University of Washington, Seattle, WA 98195, USA
* Author for correspondence (e-mail: jpriess{at}fhcrc.org)
Accepted 19 July 2004
Human ataxin 2 is a protein of unknown function that is implicated in the neurodegenerative disorder spinocerebellar ataxia type 2. We found that the C. elegans ortholog of ataxin 2, ATX-2, forms a complex with PAB-1, a cytoplasmic poly(A)-binding protein, and that ATX-2 is required for development of the germline. In the absence of ATX-2, proliferation of stem cells is reduced, and the germline is abnormally masculinized. These defects appear to result from inappropriate translational regulation that normally is mediated by the conserved KH-domain protein GLD-1. We find that MEX-3, a second KH-domain protein, exhibits a novel, ATX-2-dependent role in preventing inappropriate translation in the germline stem cells. Together, our results suggest that ATX-2 functions in translational regulation that is mediated by GLD-1 and MEX-3 proteins.
Key words: ATX-2, GLD-1, MEX-3, PAB-1, Germline, Translational regulation
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