@article{Hansson2003Overe-7642,
  year={2003},
  doi={10.1016/S0006-8993(02)04275-0},
  title={Overexpression of heat shock protein 70 in R6 / 2 Huntington s disease mice has only modest effects on disease progression},
  number={1/2},
  volume={970},
  issn={0006-8993},
  journal={Brain Research},
  pages={47--57},
  author={Hansson, Oskar and Nylandsted, Jesper and Castilho, Roger F. and Leist, Marcel and Jäättelä, Marja and Brundin, Patrik}
}

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    <dc:creator>Nylandsted, Jesper</dc:creator>
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    <dcterms:title>Overexpression of heat shock protein 70 in R6 / 2 Huntington s disease mice has only modest effects on disease progression</dcterms:title>
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    <dcterms:abstract xml:lang="eng">Huntington s disease (HD) is a neurodegenerative disorder caused by expansion of a polyglutamine tract in a protein called huntingtin. The inducible form of heat shock protein 70 (Hsp70) has been shown to reduce polyglutamine-induced toxicity. To investigate if overexpression of Hsp70 can affect disease progression in a mouse model of HD, we crossed R6 / 2 mice, expressing exon 1 of the HD gene with an expanded CAG repeat, with mice overexpressing Hsp70 (both types of transgenic mice were of the CBAxC57BL / 6 strain). The resulting R6 / 2-Hsp70 transgenics exhibited 5- to 15-fold increases in Hsp70 expression in neocortical, hippocampal and basal ganglia regions. This correlated with a delayed loss of body weight compared to R6 / 2 mice. However, the number or size of nuclear inclusions, the loss of brain weight, reduction of striatal volume, reduction in size of striatal projection neurons, downregulation of DARPP-32, development of paw clasping phenotype and early death of the mice were not affected by Hsp70 overexpression. Interestingly, the polyglutamine protein affected the potential rescuing agent, because in older R6 / 2-Hsp70 mice a large proportion of the Hsp70 protein was sequestrated in nuclear inclusions.</dcterms:abstract>
    <dc:contributor>Brundin, Patrik</dc:contributor>
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    <dc:creator>Jäättelä, Marja</dc:creator>
    <dc:creator>Hansson, Oskar</dc:creator>
    <dc:contributor>Jäättelä, Marja</dc:contributor>
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    <dc:date rdf:datatype="http://www.w3.org/2001/XMLSchema#dateTime">2011-03-24T17:36:00Z</dc:date>
    <dc:creator>Leist, Marcel</dc:creator>
    <dc:contributor>Nylandsted, Jesper</dc:contributor>
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    <dcterms:bibliographicCitation>First publ. in: Brain Research 970 (2003), 1/2, pp. 47-57</dcterms:bibliographicCitation>
    <dc:contributor>Castilho, Roger F.</dc:contributor>
    <dc:creator>Brundin, Patrik</dc:creator>
    <dc:contributor>Leist, Marcel</dc:contributor>
    <dc:contributor>Hansson, Oskar</dc:contributor>
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