| http://purl.uniprot.org/citations/22570635 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/22570635 | http://www.w3.org/2000/01/rdf-schema#comment | "Fragile X-associated tremor/ataxia syndrome (FXTAS), a late-onset neurodegenerative disorder, has been recognized in older male fragile X premutation carriers and is uncoupled from fragile X syndrome. Using a Drosophila model of FXTAS, we previously showed that transcribed premutation repeats alone are sufficient to cause neurodegeneration. MiRNAs are sequence-specific regulators of post-transcriptional gene expression. To determine the role of miRNAs in rCGG repeat-mediated neurodegeneration, we profiled miRNA expression and identified selective miRNAs, including miR-277, that are altered specifically in Drosophila brains expressing rCGG repeats. We tested their genetic interactions with rCGG repeats and found that miR-277 can modulate rCGG repeat-mediated neurodegeneration. Furthermore, we identified Drep-2 and Vimar as functional targets of miR-277 that could modulate rCGG repeat-mediated neurodegeneration. Finally, we found that hnRNP A2/B1, an rCGG repeat-binding protein, can directly regulate the expression of miR-277. These results suggest that sequestration of specific rCGG repeat-binding proteins could lead to aberrant expression of selective miRNAs, which may modulate the pathogenesis of FXTAS by post-transcriptionally regulating the expression of specific mRNAs involved in FXTAS."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pgen.1002681"xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/author | "Chen D."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/author | "Li H."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/author | "Jin P."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/author | "Tan H."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/author | "Poidevin M."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/date | "2012"xsd:gYear |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/name | "PLoS Genet"xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/pages | "e1002681"xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/title | "MicroRNA-277 modulates the neurodegeneration caused by Fragile X premutation rCGG repeats."xsd:string |
| http://purl.uniprot.org/citations/22570635 | http://purl.uniprot.org/core/volume | "8"xsd:string |
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