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

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/67985904:_____/15:00452791

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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.

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

FH - Neurologie, neurochirurgie, neurovědy

OECD FORD obor

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Projekt

Výsledek vznikl pri realizaci vícero projektů. Více informací v záložce Projekty.

Návaznosti

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Rok uplatnění

2015

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Česká a Slovenská neurologie a neurochirurgie

ISSN

1210-7859

e-ISSN

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Svazek periodika

78

Číslo periodika v rámci svazku

Suppl 2

Stát vydavatele periodika

CZ - Česká republika

Počet stran výsledku

4

Strana od-do

66-69

Kód UT WoS článku

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EID výsledku v databázi Scopus

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