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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

From: RNA dependent suppression of C9orf72 ALS/FTD associated neurodegeneration by Matrin-3

Fig. 4

RRM2 domain of MATR3 required to mediate G4C2-HRE toxicity in vivo. a Schematic of 847 amino acid (aa)-long MATR3 protein and its functional domains: two RNA-recognition motifs (RRM1/2) and two zinc-finger domains (ZF1/2). b Schematic of MATR3 with each functional domain deleted to generate deletion mutants: ΔRRM1, ΔRRM2, ΔZF1, ΔZF2. c Representative images of Drosophila eyes from flies co-expressing G4C2-30R with MATR3 (full-length) or deletion variants (ΔRRM1, ΔRRM2, ΔZF1, ΔZF2). Zoom panels emphasize the degree of ommatidial disorganization and depigmentation. MATR3-ΔRRM2 suppresses G4C2-30R toxicity to a lesser degree compared to MATR3 wildtype, indicated by increased degenerative phenotypes including de-pigmentation and ommatidial fusion compared to G4C2-30R + MATR3 (full-length). d Quantification of eye degeneration revealed no difference in G4C2-30R + MATR3-ΔRRM2 compared to G4C2-30R alone (Kruskal–Wallis test) n ≥ 50 per genotype e Kaplan–Meier survival curve of adult flies showed that neuronal expression of MATR3-ΔRRM2 mutant in G4C2-30R flies did not modify reduced survival defect in G4C2-30R flies (Log Rank Mantel Cox test) n = 100 flies per genotype. Error bars indicate S.E.M. *p value < 0.05, ****p value < 0.001

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