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, Histologie Expérimentale de Montpellier, the imaging facility MRI, member of the national infrastructure France-BioImaging supported by the French National Research Agency (ANR-10-INBS-04, ?Investments for the future?)and the INM animal facility for their services. A.M.S. was a recipient of NUMEV grant. is work was supported by grants from the Institut National de la Santé et de la Recherche Médicale (Inserm), the Association française pour la Recherche sur la SLA (ARSLA), ANR Mimetic

C. G. and F. S. , conceived the experiments. C.G. conducted the experiments. F.S. performed the qPCR and immunocytochemistry experiments and prepared the corresponding figures. B.V. performed the AFM measurements

M. , performed genotyping, cell culturing and di erentiation of myotubes. F.C. and C.R. provided advice during data analysis and contributed to manuscript writing