Publikation: Overexpression of heat shock protein 70 in R6 / 2 Huntington s disease mice has only modest effects on disease progression
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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.
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HANSSON, Oskar, Jesper NYLANDSTED, Roger F. CASTILHO, Marcel LEIST, Marja JÄÄTTELÄ, Patrik BRUNDIN, 2003. Overexpression of heat shock protein 70 in R6 / 2 Huntington s disease mice has only modest effects on disease progression. In: Brain Research. 2003, 970(1/2), pp. 47-57. ISSN 0006-8993. Available under: doi: 10.1016/S0006-8993(02)04275-0BibTex
@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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