The Rod-derived Cone Viability bi-functional Signalling, a potential link between environmental insults and endogenous neuroprotection

old_uid11487
titleThe Rod-derived Cone Viability bi-functional Signalling, a potential link between environmental insults and endogenous neuroprotection
start_date2012/06/07
schedule09h30
onlineno
location_infosalle de conférence Magendie
detailsDans le cadre du DU de Bertrand Bloch et François Tison, maladies neurodegeneratives.
summaryThe identification of one of the mechanisms leading to a vision loss in patients suffering from inherited degenerative retinal disorders reveals a novel family of signalling genes involved in the regulation of trophic interactions and response to oxidative stress. It represents a potential therapy for these currently untreatable diseases. The Rod-derived Cone Viability Factors are encoded by the Nucleoredoxin-like (Nxnl) genes and both the Nxnl1-/- and the Nxnl2-/- mice experience a progressive loss of photoreceptor cells. In addition, the Nxnl2-/- mouse experiences a progressive loss of olfactory function suggesting that this novel signalling extends outside the eye. These genes encode for a trophic protein whose role is to maintain the function and consequently the viability of cone photoreceptors. Interestingly, they also encode by differential splicing for second protein products that have the characteristics of thioredoxin-like enzyme and protects the photoreceptors and more specifically its interacting protein partner, the TAU protein, against oxidative damage. Biographical Sketch (Thierry Léveillard) I obtained my PhD at the University of Rouen (France) in 1989, following a Masters in Biochemistry at the University Pierre and Marie Curie in Paris. I spent three years as a post-doctoral researcher in San Diego (UCSD and the Salk Institute) and moved back to Strasbourg where I worked at the IGBMC for four years. My current project was initiated in Strasbourg where I was recruited as ‘Chargé de recherché Inserm’ in 1998 and ultimately continued in Paris where I was promoted ‘Directeur de recherche’ in 2006. The identification of this novel member of the thioredoxin family, which we published in 2004 in Nature Genetics, was awarded in 2005 by the Foundation Fighting Blindness. I participated in the creation of the company Fovea-Pharmaceuticals in 2005 to valorise our high content screening expertise for the identification of novel molecules for the treatment of retinal diseases. This company was sold to Sanofi-Aventis in 2009.
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