| pubmed-article:11030754 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0025936 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0035298 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0007765 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0027746 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0752125 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0205263 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C1515926 | lld:lifeskim |
| pubmed-article:11030754 | lifeskim:mentions | umls-concept:C0384782 | lld:lifeskim |
| pubmed-article:11030754 | pubmed:issue | 17 | lld:pubmed |
| pubmed-article:11030754 | pubmed:dateCreated | 2000-11-3 | lld:pubmed |
| pubmed-article:11030754 | pubmed:abstractText | Among the eight progressive neurodegenerative diseases caused by polyglutamine expansions, spinocerebellar ataxia type 7 (SCA7) is the only one to display degeneration in both brain and retina. We show here that mice overexpressing full-length mutant ataxin-7[Q90] either in Purkinje cells or in rod photoreceptors have deficiencies in motor coordination and vision, respectively. In both models, although with different time courses, an N-terminal fragment of mutant ataxin-7 accumulates into ubiquitinated nuclear inclusions that recruit a distinct set of chaperone/proteasome subunits. A severe degeneration is caused by overexpression of ataxin-7[Q90] in rods, whereas a similar overexpression of normal ataxin-7[Q10] has no obvious effect. The degenerative process is not limited to photoreceptors, showing secondary alterations of post-synaptic neurons. These findings suggest that proteolytic cleavage of mutant ataxin-7 and trans-neuronal responses are implicated in the pathogenesis of SCA7. | lld:pubmed |
| pubmed-article:11030754 | pubmed:language | eng | lld:pubmed |
| pubmed-article:11030754 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:11030754 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11030754 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:11030754 | pubmed:month | Oct | lld:pubmed |
| pubmed-article:11030754 | pubmed:issn | 0964-6906 | lld:pubmed |
| pubmed-article:11030754 | pubmed:author | pubmed-author:MandelJ LJL | lld:pubmed |
| pubmed-article:11030754 | pubmed:author | pubmed-author:Landwehrmeyer... | lld:pubmed |
| pubmed-article:11030754 | pubmed:author | pubmed-author:SahelJ AJA | lld:pubmed |
| pubmed-article:11030754 | pubmed:author | pubmed-author:PicaudSS | lld:pubmed |
| pubmed-article:11030754 | pubmed:author | pubmed-author:YvertGG | lld:pubmed |
| pubmed-article:11030754 | pubmed:author | pubmed-author:LindenbergK... | lld:pubmed |
| pubmed-article:11030754 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:11030754 | pubmed:day | 12 | lld:pubmed |
| pubmed-article:11030754 | pubmed:volume | 9 | lld:pubmed |
| pubmed-article:11030754 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:11030754 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:11030754 | pubmed:pagination | 2491-506 | lld:pubmed |
| pubmed-article:11030754 | pubmed:dateRevised | 2008-11-21 | lld:pubmed |
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| pubmed-article:11030754 | pubmed:meshHeading | pubmed-meshheading:11030754... | lld:pubmed |
| pubmed-article:11030754 | pubmed:year | 2000 | lld:pubmed |
| pubmed-article:11030754 | pubmed:articleTitle | Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice. | lld:pubmed |
| pubmed-article:11030754 | pubmed:affiliation | Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS/INSERM/ULP, BP 163, 67404 Illkirch cedex, CU de Strasbourg, France. | lld:pubmed |
| pubmed-article:11030754 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:11030754 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:246103 | entrezgene:pubmed | pubmed-article:11030754 | lld:entrezgene |
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