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Development ePress online publication date 1 Sep 2004
doi: 10.1242/dev.01352

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Research article

ATX-2, the C. elegans ortholog of ataxin 2, functions in translational regulation in the germline


Rafal Ciosk, Michael DePalma, and James R. Priess*
* Author for correspondence (e-mail: jpriess{at}fhcrc.org)

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.
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