| pubmed-article:17189700 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C1522424 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0026336 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0162508 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0035309 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0521390 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0752125 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0277785 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C0205421 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C1879547 | lld:lifeskim |
| pubmed-article:17189700 | lifeskim:mentions | umls-concept:C1292733 | lld:lifeskim |
| pubmed-article:17189700 | pubmed:issue | 3 | lld:pubmed |
| pubmed-article:17189700 | pubmed:dateCreated | 2007-3-2 | lld:pubmed |
| pubmed-article:17189700 | pubmed:abstractText | We have approached the role of cellular stress in neurodegenerative diseases caused by polyglutamine expansion (polyQ) in the context of Spinocerebellar ataxia type 7 (SCA7) that includes retinal degeneration. Using the R7E mouse, in which polyQ-ataxin-7 is specifically over-expressed in rod photoreceptors, we previously showed that rod dysfunction correlated to moderate and prolonged activation of the JNK/c-Jun stress pathway. SCA7 retinopathy was also associated with reduced expression of rod-specific genes, including the transcription factor Nrl, which is essential for rod differentiation and function. Here, we report that R7E retinopathy is improved upon breeding with the JunAA knock-in mice, in which JNK-mediated activation of c-Jun is compromised. Expression of Nrl and its downstream targets, which are involved in phototranduction, are partially restored in the JunAA-R7E mice. We further show that c-Jun can directly repress the transcription of Nrl. Our studies suggest that polyQ-induced cellular stress leads to repression of genes necessary for neuronal fate and function. | lld:pubmed |
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| pubmed-article:17189700 | pubmed:language | eng | lld:pubmed |
| pubmed-article:17189700 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:17189700 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:17189700 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:17189700 | pubmed:month | Mar | lld:pubmed |
| pubmed-article:17189700 | pubmed:issn | 0969-9961 | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:SwaroopAnandA | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:AkimotoMasayu... | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:TrottierYvonY | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:WeberChantalC | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:MerienneKarin... | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:Abou-Sleymane... | lld:pubmed |
| pubmed-article:17189700 | pubmed:author | pubmed-author:FriedmanJames... | lld:pubmed |
| pubmed-article:17189700 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:17189700 | pubmed:volume | 25 | lld:pubmed |
| pubmed-article:17189700 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:17189700 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:17189700 | pubmed:pagination | 571-81 | lld:pubmed |
| pubmed-article:17189700 | pubmed:dateRevised | 2009-11-19 | lld:pubmed |
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| pubmed-article:17189700 | pubmed:year | 2007 | lld:pubmed |
| pubmed-article:17189700 | pubmed:articleTitle | Preventing polyglutamine-induced activation of c-Jun delays neuronal dysfunction in a mouse model of SCA7 retinopathy. | lld:pubmed |
| pubmed-article:17189700 | pubmed:affiliation | Department of Molecular Pathology, Institut de Génétique et Biologie Moléculaire et Cellulaire, CNRS/INSERM/ULP, BP10142, 67404 Illkirch Cédex, CU de Strasbourg, France. merienne@igbmc.u-strasbg.fr | lld:pubmed |
| pubmed-article:17189700 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:17189700 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| pubmed-article:17189700 | pubmed:publicationType | Research Support, N.I.H., Extramural | lld:pubmed |
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| http://linkedlifedata.com/r... | entrezgene:pubmed | pubmed-article:17189700 | lld:entrezgene |
