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Typage moléculaire des maladies neurodégénératives dues à l’agrégation de la protéine alpha synucléine

Alexis Fenyi 1
1 LMN - Laboratoire des Maladies Neurodégénératives - UMR 9199
MIRCEN - Service MIRCEN : DRF/JACOB, CNRS - Centre National de la Recherche Scientifique : UMR 9199
Abstract : The aggregation of α-synuclein protein has been shown to be associated with Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy, called synucleinopathies. Increasing amount of evidences suggest that synucleinopathies are prion diseases. Some aspects are missing for α-synuclein to be recognized as a prion, such as the existence of strains associated to synucleinopathies. During my thesis I set up a reliable method to amplify α-synuclein-rich deposits from patients tissues. I validated the method using all synucleinopathies tissues. This should allow the identification of α-synuclein strain related to each synucleinopathy. In addition, I also documented cleaning procedures for materials soiled with various amyloid fibers, in order to reduce the risk of contamination. Finally, I was associated to a study that shows the propagation abilities of different α-synuclein assemblies in a neuronal network mimicking human cortico-cortical connections. These results open the way to structural and functional studies of the amplified deposits.
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Alexis Fenyi. Typage moléculaire des maladies neurodégénératives dues à l’agrégation de la protéine alpha synucléine. Biologie structurale [q-bio.BM]. Université Paris Saclay (COmUE), 2019. Français. ⟨NNT : 2019SACLS053⟩. ⟨tel-02476810v2⟩

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