Additional file 4 of RNA dependent suppression of C9orf72 ALS/FTD associated neurodegeneration by Matrin-3

MATR3-mediated suppression G4C2-30R toxicity retained upon aging. (A) Representative images of Drosophila eyes from flies expressing G4C2-30R along with UAS-EGFP (control for GAL4 dilution), UAS-MATR3, or (B) UAS-MATR3-ΔRRM2. Flies expressing G4C2-30R develop exacerbated eye degeneration upon aging, indicated by increase in de-pigmentation, ommatidial fusion and development of new necrotic patches. Ectopic expression of MATR3 continued to strongly suppress eye degeneration upon aging. Ectopic expression of MATR3-ΔRRM2 also suppressed eye degeneration in G4C2-30R flies at 30-days, however to a much lesser extent compared to full-length MATR3. (C) Quantification of external eye degeneration showed statistically significant suppression of G4C2-30R- mediated eye degeneration upon MATR3 expression, and to a milder extent, upon MATR3-ΔRRM2 expression (Kruskal–Wallis test) n ≥ 50 flies per genotype. Error bars indicate S.E.M. ****p value < 0.0001