Ca2+/recoverin dependent regulation of phosphorylation of the rhodopsin mutant R135L associated with retinitis pigmentosa |
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Authors: | Senin Ivan I Bosch Laia Ramon Eva Zernii Evgeni Yu Manyosa Joan Philippov Pavel P Garriga Pere |
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Institution: | Department of Cell Signalling, A. N. Belozersky Institute of Physico-Chemical Biology, M.V. Lomonosov Moscow State University, Russia. |
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Abstract: | No single molecular mechanism accounts for the effect of mutations in rhodopsin associated with retinitis pigmentosa. Here we report on the specific effect of a Ca2+/recoverin upon phosphorylation of the autosomal dominant retinitis pigmentosa R135L rhodopsin mutant. This mutant shows specific features like impaired G-protein signaling but enhanced phosphorylation in the shut-off process. We now report that R135L hyperphosphorylation by rhodopsin kinase is less efficiently inhibited by Ca2+/recoverin than wild-type rhodopsin. This suggests an involvement of Ca2+/recoverin into the molecular pathogenic effect of the mutation in retinitis pigmentosa which is the cause of rod photoreceptor cell degeneration. This new proposed role of Ca2+/recoverin may be one of the specific features of the proposed new Type III class or rhodopsin mutations associated with retinitis pigmentosa. |
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Keywords: | Rhodopsin phosphorylation Recoverin Calcium Rhodopsin mutation Retinitis pigmentosa Rhodopsin kinase Transducin activation Visual phototransduction |
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