Rod-derived cone viability factor for treating blinding diseases: from clinic to redox signaling.
The identification of one mechanism that causes vision loss in inherited degenerative retinal disorders revealed a new signaling molecule that represents a potential therapy for these currently untreatable diseases. This protein, called rod-derived cone viability factor (RdCVF), maintains the function and consequently the viability of cone photoreceptor cells in the retina; mice that lack this factor exhibit a progressive loss of photoreceptor cells. The gene encoding RdCVF also encodes, by differential splicing, a second product that has characteristics of a thioredoxin-like enzyme and protects both photoreceptor cells and, more specifically, its interacting protein partner, the tau protein, against oxidative damage. This signaling pathway potentially links environmental insults to an endogenous neuroprotective response.
Léveillard
Thierry
T
Department of Genetics, Institut de la Vision, INSERM, UPMC University of Paris 06, UMR-S 968, CNRS 7210, Paris F-75012, France. thierry.leveillard@inserm.fr
Sahel
José-Alain
JA
D000818:Animals; D001766:Blindness / Q000628:therapy*; D002470:Cell Survival; D006801:Humans; D051379:Mice; D010084:Oxidation-Reduction; D018384:Oxidative Stress; D017949:Retinal Cone Photoreceptor Cells / Q000378:metabolism*; D012162:Retinal Degeneration / Q000628:therapy; D012164:Retinal Diseases / Q000628:therapy; D017948:Retinal Rod Photoreceptor Cells / Q000378:metabolism*; D015398:Signal Transduction; D013879:Thioredoxins / Q000235:genetics / Q000378:metabolism*; D057170:Translational Research, Biomedical
D013879:Thioredoxins