Abstract
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 polyA-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.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, Non-P.H.S.
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Animals
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Ataxins
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Caenorhabditis elegans / genetics
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Caenorhabditis elegans / growth & development
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Caenorhabditis elegans / metabolism
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Caenorhabditis elegans Proteins / genetics
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Caenorhabditis elegans Proteins / metabolism*
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Female
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Germ Cells / metabolism
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Humans
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Male
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Membrane Proteins / genetics
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Membrane Proteins / metabolism
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Nerve Tissue Proteins
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Oogenesis
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Poly(A)-Binding Proteins / metabolism
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Protein Biosynthesis
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Proteins / genetics
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Proteins / metabolism*
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RNA Interference
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RNA, Helminth / genetics
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RNA, Helminth / metabolism
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RNA, Messenger / genetics
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RNA, Messenger / metabolism
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RNA-Binding Proteins / metabolism
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Spinocerebellar Ataxias / genetics
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Spinocerebellar Ataxias / metabolism
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Stem Cells / metabolism
Substances
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Ataxins
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Caenorhabditis elegans Proteins
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GLD-1 protein, C elegans
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MEX-3 protein, C elegans
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Membrane Proteins
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Nerve Tissue Proteins
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Poly(A)-Binding Proteins
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Proteins
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RNA, Helminth
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RNA, Messenger
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RNA-Binding Proteins
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tra-2 protein, C elegans