Longitudinal study revealing motor, cognitive and behavioral decline in a transgenic minipig model of Huntington's disease

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985904%3A_____%2F20%3A00523986" target="_blank" >RIV/67985904:_____/20:00523986 - isvavai.cz</a>

Result on the web

<a href="https://dmm.biologists.org/content/13/2/dmm041293" target="_blank" >https://dmm.biologists.org/content/13/2/dmm041293</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1242/dmm.041293" target="_blank" >10.1242/dmm.041293</a>

Result language

angličtina

Original language name

Longitudinal study revealing motor, cognitive and behavioral decline in a transgenic minipig model of Huntington's disease

Original language description

Huntington's disease (HD) is an inherited devastating neurodegenerative disease with no known cure to date. Several therapeutic treatments for HD are in development, but their safety, tolerability and efficacy need to be tested before translation to bedside. The monogenetic nature of this disorder has enabled the generation of transgenic animal models carrying a mutant huntingtin (mHTT) gene causing HD. A large animal model reflecting disease progression in humans would be beneficial for testing the potential therapeutic approaches. Progression of the motor, cognitive and behavioral phenotype was monitored in transgenic Huntington's disease minipigs (TgHD) expressing the N-terminal part of human mHTT. New tests were established to investigate physical activity by telemetry, and to explore the stress-induced behavioral and cognitive changes in minipigs. The longitudinal study revealed significant differences between 6- to 8-year-old TgHD animals and their wild-type (WT) controls in a majority of the tests. The telemetric study showed increased physical activity of 4.6- to 6.5-year-old TgHD boars compared to their WT counterparts during the lunch period as well as in the afternoon. Our phenotypic study indicates progression in adult TgHD minipigs and therefore this model could be suitable for longstanding preclinical studies of HD.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

10601 - Cell biology

Project

Result was created during the realization of more than one project. More information in the Projects tab.

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2020

Confidentiality

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

Name of the periodical

Disease Models & Mechanisms

ISSN

1754-8411

e-ISSN

—

Volume of the periodical

13

Issue of the periodical within the volume

2

Country of publishing house

GB - UNITED KINGDOM

Number of pages

9

Pages from-to

dmm041293

UT code for WoS article

000518475500007

EID of the result in the Scopus database

2-s2.0-85076873575