| pubmed-article:15829505 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C0020179 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C0025914 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C0026336 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C0234131 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C0038952 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C1415504 | lld:lifeskim |
| pubmed-article:15829505 | lifeskim:mentions | umls-concept:C1517945 | lld:lifeskim |
| pubmed-article:15829505 | pubmed:issue | 10 | lld:pubmed |
| pubmed-article:15829505 | pubmed:dateCreated | 2005-5-13 | lld:pubmed |
| pubmed-article:15829505 | pubmed:abstractText | Huntington disease (HD) is an adult-onset neurodegenerative disease caused by a toxic gain of function in the huntingtin (htt) protein. The contribution of wild-type htt function to the pathogenesis of HD is currently uncertain. To assess the role of wild-type htt in HD, we generated YAC128 mice that do not express wild-type htt (YAC128-/-) but express the same amount of mutant htt as normal YAC128 mice (YAC128+/+). YAC128-/- mice perform worse than YAC128+/+ mice in the rotarod test of motor coordination (P = 0.001) and are hypoactive compared with YAC128+/+ mice at 2 months (P = 0.003). Striatal neuropathology was not clearly worse in YAC128-/- mice compared with YAC128+/+ mice. There was no significant effect of decreased wild-type htt on striatal volume, neuronal counts or DARPP-32 expression but a modest worsening of striatal neuronal atrophy was evident (6%, P = 0.03). The testis of YAC128+/+ mice showed atrophy and degeneration, which was markedly worsened in the absence of wild-type htt (P = 0.001). YAC128+/+ mice also showed a male specific deficit in survival compared with WT mice which was exacerbated by the loss of wild-type htt (12-month-male survival, P < 0.001). Overall, we demonstrate that the loss of wild-type htt influences motor dysfunction, hyperkinesia, testicular degeneration and impaired lifespan in YAC128 mice. The mild effect of wild-type htt on striatal phenotypes in YAC128 mice suggests that the characteristic striatal neuropathology in HD is caused primarily by the toxicity of mutant htt and that replacement of wild-type htt will not be an adequate treatment for HD. | lld:pubmed |
| pubmed-article:15829505 | pubmed:language | eng | lld:pubmed |
| pubmed-article:15829505 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:15829505 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:15829505 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:15829505 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:15829505 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:15829505 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:15829505 | pubmed:month | May | lld:pubmed |
| pubmed-article:15829505 | pubmed:issn | 0964-6906 | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:HaydenMichael... | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:LeavittBlair... | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:VoglA WayneAW | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:BissadaNagatN | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:RogersDaniel... | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:Van... | lld:pubmed |
| pubmed-article:15829505 | pubmed:author | pubmed-author:PearsonJacque... | lld:pubmed |
| pubmed-article:15829505 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:15829505 | pubmed:day | 15 | lld:pubmed |
| pubmed-article:15829505 | pubmed:volume | 14 | lld:pubmed |
| pubmed-article:15829505 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:15829505 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:15829505 | pubmed:pagination | 1379-92 | lld:pubmed |
| pubmed-article:15829505 | pubmed:dateRevised | 2006-11-15 | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:meshHeading | pubmed-meshheading:15829505... | lld:pubmed |
| pubmed-article:15829505 | pubmed:year | 2005 | lld:pubmed |
| pubmed-article:15829505 | pubmed:articleTitle | Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease. | lld:pubmed |
| pubmed-article:15829505 | pubmed:affiliation | Department of Medical Genetics, University of British Columbia, Vancouver, Canada. | lld:pubmed |
| pubmed-article:15829505 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:15829505 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:15194 | entrezgene:pubmed | pubmed-article:15829505 | lld:entrezgene |
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