Titre : | Modification of proteotoxicity associated to neuromuscular diseases : genetic analysis in C. elegans |
contenu dans : | |
Auteurs : | Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Pasco M ; Catoire H ; Tourette C ; Parker A ; Néri C |
Type de document : | Article |
Année de publication : | 2008 |
Pages : | p. 408 |
Langues: | Anglais |
Résumé : | The goal of our study is to identify signalling pathways and networks that may modify proteotoxicity associated to neuromuscular diseases. The rationale of our study is that genetic/biological modifiers of proteotoxicity may constitue a useful source of disease targets and markers. We use a combination of genetic and pharmacological manipulations to test whether genes and compounds may modify the cytotoxicity produced by disease protein expression in transgenic nematodes. Results will be presented showing that disease protein expression produces cellular and behavioral defects in C. elegans transgenics and allows conserved modulators of proteotoxicity to be identified, some of them which may be manipulated by pharmacological means. Our data suggest that C. elegans genetics may be a useful translational research component and starting point to screen for neuromuscular disease targets by searching for in vivo modifiers of proteotoxicity. |