Springer Nature
40478_2020_1060_MOESM4_ESM.tif (15.03 MB)

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

Download (15.03 MB)
posted on 2020-11-01, 04:44 authored by Nandini Ramesh, Elizabeth L. Daley, Amanda M. Gleixner, Jacob R. Mann, Sukhleen Kour, Darilang Mawrie, Eric N. Anderson, Julia Kofler, Christopher J. Donnelly, Evangelos Kiskinis, Udai Bhan Pandey
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