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Huntingtine et développement cortical

Abstract : Huntington disease (HD) is an autosomal dominant inherited neurological disorder conducting to the appearance of motors, psychiatrics and cognitives symptoms during mid-adulthood. HD is characterised by a massive neurodegenerescence of both striatal and cortical neurons. HD is caused by a mutation in coding region of the protein Huntingtin (HTT) leading to the production of a mutated form (mHTT). mHTT gain new toxic function but also loss some of normal function of HTT. Therefore, studying both gain and loss of function is mandatory to better understand the physiopathological progression of HD.HTT and mHTT both contribute to development of cerebral structures. Our hypothesis is that developmental defects induced by mHTT could contribute at least in part to the physiological progression of HD. Our work focuses on cerebral cortex development a structure which is largely impacted in HD. Our previous studies demonstrated roles of HTT and the effect of mHTT in neuronal precursor proliferation during neurogenesis. However, roles and functions of HTT and mHTT during later step of cortical neurogenesis remain elusive.My PhD project has focused on two main aspects: (i) study the function of HTT in newborn post-mitotic neurons in cerebral cortex, notably during their migration and maturation, and (ii), characterising cortical neurogenesis in genetically integrated mouse model of HD: zQ175.
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Submitted on : Wednesday, November 20, 2019 - 3:12:46 PM
Last modification on : Friday, March 25, 2022 - 9:43:41 AM


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  • HAL Id : tel-02372650, version 1




Julien Le Friec. Huntingtine et développement cortical. Neurosciences [q-bio.NC]. Université Grenoble Alpes, 2019. Français. ⟨NNT : 2019GREAV016⟩. ⟨tel-02372650⟩



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