Počet záznamů: 1
Different Forms of Huntingtin in the Most Affected Organs; Brain and Testes of Transgenic Minipigs
- 1.0452791 - ÚŽFG 2016 RIV CZ eng J - Článek v odborném periodiku
Vidinská, Daniela - Motlík, Jan - Ellederová, Zdeňka
Different Forms of Huntingtin in the Most Affected Organs; Brain and Testes of Transgenic Minipigs.
Česká a Slovenská neurologie a neurochirurgie. Roč. 78, Suppl 2 (2015), s. 66-69. ISSN 1210-7859. E-ISSN 1802-4041.
[Conference on Animal Models for neurodegenerative Diseases /3./. Liblice, 08.11.2015-10.11.2015]
Grant CEP: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308
Institucionální podpora: RVO:67985904
Klíčová slova: Huntington´s disease * transgenic minipig model * mutant huntingtin
Kód oboru RIV: FH - Neurologie, neurochirurgie, neurovědy
Impakt faktor: 0.209, rok: 2015
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.
Trvalý link: http://hdl.handle.net/11104/0253764
Počet záznamů: 1