title: ATX-2, the C. elegans ortholog of ataxin 2, functions in translational regulation in the germline.
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.
authors.affiliation: Division of Basic Sciences, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA.
authors.forename: Rafal
authors.initials: R
authors.lastname: Ciosk
authors.forename: Michael
authors.initials: M
authors.lastname: DePalma
authors.forename: James R
authors.initials: JR
authors.lastname: Priess
mesh_terms: D000818:Animals; D000067528:Ataxins; D017173:Caenorhabditis elegans / Q000235:genetics / Q000254:growth & development / Q000378:metabolism; D029742:Caenorhabditis elegans Proteins / Q000235:genetics / Q000378:metabolism*; D005260:Female; D005854:Germ Cells / Q000378:metabolism; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism; D009419:Nerve Tissue Proteins; D009866:Oogenesis; D039102:Poly(A)-Binding Proteins / Q000378:metabolism; D014176:Protein Biosynthesis; D011506:Proteins / Q000235:genetics / Q000378:metabolism*; D034622:RNA Interference; D018510:RNA, Helminth / Q000235:genetics / Q000378:metabolism; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D016601:RNA-Binding Proteins / Q000378:metabolism; D020754:Spinocerebellar Ataxias / Q000235:genetics / Q000378:metabolism; D013234:Stem Cells / Q000378:metabolism
chemical_list: D000067528:Ataxins; D029742:Caenorhabditis elegans Proteins; C094448:GLD-1 protein, C elegans; C102175:MEX-3 protein, C elegans; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D018510:RNA, Helminth; D012333:RNA, Messenger; D016601:RNA-Binding Proteins; C076107:tra-2 protein, C elegans