Large Animal Models of Huntington's Disease: What We Have Learned and Where We Need to Go Next

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985904%3A_____%2F20%3A00537797" target="_blank" >RIV/67985904:_____/20:00537797 - isvavai.cz</a>
Result on the web
<a href="https://content.iospress.com/articles/journal-of-huntingtons-disease/jhd200425" target="_blank" >https://content.iospress.com/articles/journal-of-huntingtons-disease/jhd200425</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.3233/JHD-200425" target="_blank" >10.3233/JHD-200425</a>

Result language
angličtina
Original language name
Large Animal Models of Huntington's Disease: What We Have Learned and Where We Need to Go Next
Original language description
Genetically modified rodent models of Huntington's disease (HD) have been especially valuable to our under-standing of HD pathology and the mechanisms by which the mutant HTT gene alters physiology. However, due to inherent differences in genetics, neuroanatomy, neurocircuitry and neurophysiology, animal models do not always faithfully or fully recapitulate human disease features or adequately predict a clinical response to treatment. Therefore, conducting translational studies of candidate HD therapeutics only in a single species (i.e. mouse disease models) may not be sufficient. Large animal models of HD have been shown to be valuable to the HD research community and the expectation is that the need for translational studies that span rodent and large animal models will grow. Here, we review the large animal models of HD that have been created to date, with specific commentary on differences between the models, the strengths and disadvantages of each, and how we can advance useful models to study disease pathophysiology, biomarker development and evaluation of promising therapeutics.
Czech name
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Czech description
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Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
10603 - Genetics and heredity (medical genetics to be 3)

Project
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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

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