José-Alain Sahel – December 16, 2011
INC Open Mind Seminar Series
Friday, December 16, 2011 at 11:30 am
Salle des Conférences (R229), Centre Universitaire des Saints-Pères, 45 rue des Saints-Pères, 75006 Paris
José-Alain Sahel, The Vision Institute
From neuroprotection to restoration of the photoreceptor function:
a translational approach
In the retinal dystrophies, multiple genetic anomalies leading to photoreceptor degeneration play causative role. Therapeutic strategies targeting directly the genes responsible for the disease are logical and have recently been shown to be effective in one of the forms of Leber congenital amaurosis.
During retinal degeneration progression, it is important to underline that the neural circuits in the inner retina are not destroyed and cone photoreceptors are saved for several years despite the lost of their outer segment and their function, from where the concept of “dormant cones”. Supposing that the partially persistent neuronal circuits are functional, the preservation and the stimulation of the still present retinal neurons open great hopes. Retinal prostheses have been successfully implanted and demonstrated to restore useful vision in blind patients by stimulating the residual neuronal circuits in the retina. The expression of channelrhodopsin or halorhodopsin in the retinal ganglion and bipolar cells or in the preserved cones restores visual function in animal models of retinal degeneration and in human retinas post-mortem.
Innovating high-resolution in vivo noninvasive imagery techniques make it possible to observe the “dormant cones” and to analyze the structure of the inner retina, allowing the identification of blind people that may benefit from these therapeutic strategies. Owing to our innovating translational approach which integrates high-resolution imagery techniques, nanotechnologies applied to retinal prostheses, genetics, optogenetics, and gene therapy, we have an exceptional ensemble of diagnostic, prognostic and therapeutic means to fight vision loss related to numerous diseases, in particular to currently incurable genetic diseases. Such a translational research implies a strong collaboration with industrial and academic partners in fields of expertise such as mathematics, physics, astronomy or bioinformatics, to offer patients the best possible diagnostic, preventive and therapeutic solutions.
Professor José-Alain Sahel is the Director of The Vision Institute, a Translational UPMC/Inserm/CNRS Research Centre, Paris, France; Professor of Ophthalmology at Pierre and Marie Curie University Medical School, and the Cumberlege Professor of Biomedical Sciences at the Institute of Ophthalmology, University College London. Professor Sahel chairs a department of Ophthalmology at the Quinze-Vingts National Ophthalmology Hospital and at the Rothschild Ophthalmology Foundation and coordinates the Ophthalmology Clinical Investigation Centre and the National Reference Centre for Retinal Dystrophies.M.D., Ph.D., Professor in Child Psychiatry, Laboratoire Psychologie de la Perception, CNRS UMR 8158 and Paris Descartes