Different Forms of Huntingtin in the Most  Affected Organs; Brain and Testes of Transgenic Minipigs

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985904%3A_____%2F15%3A00476994" target="_blank" >RIV/67985904:_____/15:00476994 - isvavai.cz</a>
Alternative codes found
RIV/67985904:_____/15:00452791
Result on the web
<a href="http://dx.doi.org/10.14735/amcsnn20152S66" target="_blank" >http://dx.doi.org/10.14735/amcsnn20152S66</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.14735/amcsnn20152S66" target="_blank" >10.14735/amcsnn20152S66</a>

Result language
angličtina
Original language name
Different Forms of Huntingtin in the Most  Affected Organs; Brain and Testes of Transgenic Minipigs
Original language description
Huntington’s disease (HD) is a neurodegenerative disorder caused by the the elongation of CAG triplet repeat in the gene encoding the huntingtin protein (Htt). In patients, in addition to the monomeric form of huntingtin, N terminal fragments, oligomers, and polymers are present mostly in the affected tissues, even though the mutated huntingtin (mtHtt) is expressed basically in all cells. The most affected tissues are basal ganglia and cerebral cortex. In this study we analyzed the presence of N terminal fragments and oligomers of Htt in different tissues of 24 and 36 months old experimental animals. This was done in our large animal model of HD, which uses transgenic (TgHD) minipigs expressing N terminal part of human mtHtt. Among all the tissues tested, we found cortex and testes to contain N terminal fragments as well as oligomeric smears in TgHD minipigs compared to wild type siblings. On the other hand, we did not detect any fragments or oligomers in muscle and heart of TgHD minipigs, only starting fragmentation in muscles of 36 months old animals. These findings mimic the early progression of the disease in humans, hence presents minipig as a promising model for therapeutic testing of HD.
Czech name
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Czech description
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Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FH - Neurology, neuro-surgery, nuero-sciences
OECD FORD branch
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Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Publication year
2015
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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