Primary fibroblasts for studying molecular mechanisms in Huntington´s disease

Kód výsledku v 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>

Výsledek na webu

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DOI - Digital Object Identifier

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Jazyk výsledku

angličtina

Název v původním jazyce

Primary fibroblasts for studying molecular mechanisms in Huntington´s disease

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Primary fibroblasts for studying molecular mechanisms in Huntington´s disease

Popis výsledku anglicky

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.

Druh

C - Kapitola v odborné knize

CEP obor

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OECD FORD obor

10608 - Biochemistry and molecular biology

Projekt

<a href="/cs/project/LO1609" target="_blank" >LO1609: Modely závažných lidských onemocnění: Traumatické poškození míchy, Huntingtonova choroba, melanom a neplodnost</a><br>

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2020

Kód důvěrnosti údajů

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

Název knihy nebo sborníku

Translational Research in Serious Human Diseases

ISBN

978-80-200-3158-7

Počet stran výsledku

7

Strana od-do

57-63

Počet stran knihy

271

Název nakladatele

Academia

Místo vydání

Praha

Kód UT WoS kapitoly

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