31P MR Spectroscopy of the Testes and Immunohistochemical Analysis of Sperm 
of Transgenic Boars Carried N terminal Part of Human Mutated Huntingtin

0476983 - ÚŽFG 2017 RIV CZ eng J - Článek v odborném periodiku
Jozefovičová, M. - Herynek, V. - Jírů, F. - Dezortová, M. - Juhásová, Jana - Juhás, Štefan - Klíma, Jiří - Bohuslavová, Božena - Motlík, Jan - Hájek, M.
31P MR Spectroscopy of the Testes and Immunohistochemical Analysis of Sperm 
of Transgenic Boars Carried N terminal Part of Human Mutated Huntingtin.
Česká a Slovenská neurologie a neurochirurgie. Roč. 78, Suppl 2 (2015), s. 28-33. 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 MŠMT(CZ) 7F14308
Klíčová slova: Huntington´s disease * testes * sperm
Kód oboru RIV: FH - Neurologie, neurochirurgie, neurovědy
Impakt faktor: 0.209, rok: 2015

Huntington’s disease (HD) is an inherited autosomal neurodegenerative disorder characterized by motor dysfunctions, behavioral and cognitive disturbances. It affects predominantly the brain, however, changes were found also in peripheral tissues. Some of these changes can result from direct expression of mutant huntingtin; its highest levels have been found in the brain and testes. In 2009 we established a minipig model of HD (TgHD) expressing N terminal (548aa) part of human mutated huntingtin encoded 124 CAG/ CAA repeats. Previous research has revealed the presence of reduced fertility and fewer spermatozoa per ejaculate in TgHD boars started at 13 months of age. The aim of this study was to determine changes in the testes of 24 months old transgenic boars (F2 generation in vivo) using non invasive methodology of 31P magnetic resonance (MR) spectroscopy as well as to perform imunohistochemical analysis of TgHD sperm collected fromform F1 and F3 generation before HD onset. The results have shown significant reduction of relative phosphodiester concentration in testicular parenchyma of TgHD boars compared to wild type (WT) ones of the same ages. Moreover immunohistochemical analysis of sperm collected from TgHD and WT have revealed exclusive anti polyQ specific (clone 3B5H10) as well as significantly increased anti huntingtin (clone EPR5526) staining in transgenic spermatozoa tails in comparison with WT counterparts. Thus, our results are suggestive of the negative impact of human mutated huntingtin on testes metabolism as well as sperm abnormalities.
Trvalý link: http://hdl.handle.net/11104/0273383