Primary fibroblasts for studying molecular mechanisms in Huntington´s disease

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985904%3A_____%2F20%3A00536501" target="_blank" >RIV/67985904:_____/20:00536501 - isvavai.cz</a>
Result on the web
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DOI - Digital Object Identifier
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Result language
angličtina
Original language name
Primary fibroblasts for studying molecular mechanisms in Huntington´s disease
Original language description
Huntington's disease (HD) is dominantly inherited neurodegenerative disorder caused by the mutation in gene encoding huntingtin protein (HTT). HTT has a role in several biological processes and thus many molecular mechanisms are affected by the mutated form of HTT (mHTT). The exact pathogenic mechanism of mHTT action in HD pathology is still not well understood. Several animal models of HD have been created. Minipigs transgenic for the N-terminal part of human mHTT showed up to be suitable large animal model of HD with the onset of locomotor and neurological impairment at the age of 48 months. Primary fibroblasts isolated from this animal model represent well and easily accessible biomaterial. They can be obtained at different ages throughout HD progression thus facilitating the investigation of molecular mechanisms in HD.
Czech name
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Czech description
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Project
<a href="/en/project/LO1609" target="_blank" >LO1609: Models of the Serious Human Diseases: Traumatic Spinal Cord Injury, Huntington’s Disease, Melanoma and Infertility</a><br>
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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