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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
4
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pubmed:dateCreated |
2007-8-16
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pubmed:abstractText |
Fragile X-associated tremor/ataxia syndrome (FXTAS) is a recently described neurodegenerative disorder of older adult carriers of premutation alleles (60-200 CGG repeats) in the fragile X mental retardation gene (FMR1). It has been proposed that FXTAS is an RNA-mediated neurodegenerative disease caused by the titration of RNA-binding proteins by the CGG repeats. To test this hypothesis, we utilize a transgenic Drosophila model of FXTAS that expresses a premutation-length repeat (90 CGG repeats) from the 5' UTR of the human FMR1 gene and displays neuronal degeneration. Here, we show that overexpression of RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppresses the phenotype of the CGG transgenic fly. Furthermore, we show that hnRNP A2/B1 directly interacts with riboCGG repeats and that the CUGBP1 protein interacts with the riboCGG repeats via hnRNP A2/B1.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-10381337,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-10631132,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-11032753,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-11124939,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-11445641,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-12076670,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-12135967,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-12700164,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-12948442,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-14671308,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-14722059,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-15052536,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-15065017,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-16239243,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-16246864,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-16332642,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-16723374,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-16776586,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-17179750,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-17698005,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-8862520,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17698010-8948631
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Aug
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pubmed:issn |
0896-6273
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
16
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pubmed:volume |
55
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
565-71
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pubmed:dateRevised |
2011-5-25
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pubmed:meshHeading |
pubmed-meshheading:17698010-Animals,
pubmed-meshheading:17698010-Animals, Genetically Modified,
pubmed-meshheading:17698010-Disease Models, Animal,
pubmed-meshheading:17698010-Drosophila,
pubmed-meshheading:17698010-Drosophila Proteins,
pubmed-meshheading:17698010-Eye,
pubmed-meshheading:17698010-Fragile X Mental Retardation Protein,
pubmed-meshheading:17698010-Fragile X Syndrome,
pubmed-meshheading:17698010-Heterogeneous-Nuclear Ribonucleoprotein Group A-B,
pubmed-meshheading:17698010-Immunoprecipitation,
pubmed-meshheading:17698010-Microscopy, Electron, Scanning,
pubmed-meshheading:17698010-Neurodegenerative Diseases,
pubmed-meshheading:17698010-RNA-Binding Proteins,
pubmed-meshheading:17698010-Trinucleotide Repeat Expansion
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pubmed:year |
2007
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pubmed:articleTitle |
RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS.
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pubmed:affiliation |
Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Research Support, N.I.H., Extramural
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