Fig. 2

SETX co-expression rescues C9orf72 and dipeptide repeat toxicity in Drosophila. a Stereomicroscopy images of representative Drosophila eyes expressing (G4C2)₅₈ driven by GMR-GAL4 reveals a rough eye phenotype (left), which is suppressed in flies concomitantly expressing normal human SETX (middle) or mutant L389S SETX (right). b Stereomicroscopy images of representative Drosophila eyes expressing protein-only GR(50) dipepetides driven by GMR-GAL4 reveals extensive eye degeneration (left), which is fully suppressed in flies concomitantly expressing normal human SETX (center), but not in flies expressing the P-loopΔ SETX mutant (right), which lacks a functional helicase catalytic domain. c We performed immunoblot analysis on protein extracts prepared from late pupal stage heads from flies of the indicated genotypes with an anti-GFP antibody. Note comparable levels of GR(50) dipeptide expression in GR(50) only flies and GR(50) / SETX(wt) flies. d We monitored survival to the adult stage for different lines of transgenic flies of the indicated genotypes, and documented nearly complete rescue of lethality in GR(50) expressing flies crossed with SETX(wt) expressing flies. Survival to the adult stage no longer occurred in GR(50) expressing flies crossed with SETX helicase domain mutation (P-loopΔ) expressing flies. Error bars = s.d