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Dynamics of Neurofilaments in health and disease| title | Dynamics of Neurofilaments in health and disease |
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| start_date | 2024/02/08 |
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| schedule | 14h |
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| online | no |
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| location_info | / |
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| summary | Neurofilaments (NFs) are the Intermediate Filaments (IFs) of the nervous system and the major cytoskeletal component of mature neurons. Previous studies have revealed that this structural network exhibits dynamics in transport and turn-over, and sustains essential cellular and physiological functions in the nervous system. Accordingly, alterations of NFs have been shown to drive neurodegeneration in human diseases. Not only are NFs a genetic cause of neuronal death in human, but their abnormal aggregation is also an early pathological hallmark of most neurodegenerative diseases, and their removal from axons has shown spectacular benefits in mouse models. With a long-lasting interest in the rare disease giant axonal neuropathy, whose mutated gene encodes for the universal regulator of IF steady-state (Gigaxonin-E3 ligase), our laboratory is exploring the neurobiology of NFs in health and disease. Our research program aims at scrutinizing the dynamics of NFs in a physiological context, the downstream signaling sustaining their essential functions and how alterations of this system can cause neurodegeneration. Here, we will first present our research on Gigaxonin to focus on our latest development in generating new tools and methodologies in the zebrafish species, whose numerous advantages constitute great assets to monitor the live imaging of NFs in vivo and to dissect their molecular signaling pathways. Finally, we will present how this knowledge will be used to offer a significant platform for therapeutic intervention in the future, for the benefit of most neurodegenerative diseases. |
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| responsibles | Perrais, Thoumine |
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Workflow history| from state (1) | to state | comment | date |
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| submitted | published | | 2024/01/16 15:40 UTC |
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