Résumé :
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To date, 3 mutations (R120G, Q151X and 464?CT) in the gene encoding ?B-crystallin have been identified as responsible of Myofibrillar Myopathies then called ?B-cristallinopathies (Selcen and Engel, 2003; Vicart et al., 1998). In order to develop a pharmacologic treatment for Myofibrillar Myopathies, we realized a large screening of a peptide aptamers library by two-hybrid method. In the preliminary results presented here, we demonstrate that aptamer strategy led us to identify several aptamers able to correct the hyper-sensitivity to oxidative stress of the R120G ?B-crystallin mutant in HeLa cells. Selcen, D., and Engel, A. G. (2003). Myofibrillar myopathy caused by novel dominant negative alpha B-crystallin mutations. Ann Neurol 54, 804-810. Vicart, P., Caron, A., Guicheney, P., Li, Z., Prevost, M. C., Faure, A., Chateau, D., Chapon, F., Tome, F., Dupret, J. M., et al. (1998). A missense mutation in the alphaB-crystallin chaperone gene causes a desmin-related myopathy. Nat Genet 20, 92-95.
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