Figure 1: CMT-mutant GARS expression shortens lifespan and induces motor performance deficits.

(a) Schematic representation of the GARS protein, with CMT-associated mutations and functional domains indicated. Mutations in blue result in loss of aminoacylation activity, mutations in green do not affect enzymatic activity and mutations in black have not been evaluated. Mutations modelled in this study are framed. (b) Kaplan–Meier survival curves displaying the lifespan of female flies ubiquitously expressing two copies of GARS transgenes from the adult stage onwards. N=182–270. (c) Bar graph displaying average climbing speed in a negative geotaxis assay of female flies expressing GARS in motor neurons (OK371-GAL4). OK371-GAL4>GARS_G240R and OK371-GAL4>2 × GARS_G526R flies displayed developmental lethality and could not be assessed. N=100. Error bars represent s.e.m. *P<0.05; ***P<1 × 10−37.