Figure 8. .
Motor neuron–selective Xrp1 knockdown mitigates motor deficits and shortened life span induced by ALS mutant FUS expression. (A and B) Life span of control (driver only) flies and flies with motor neuron–selective (D42-GAL4) expression of human FUS-R518K, Xrp1-RNAi, or both transgenes. Data for male (A) and female (B) flies are shown. n > 75 per genotype. (C and D) Average climbing speed of adult female flies with motor neuron–selective (D42-GAL4) expression of human FUS-R518K, Xrp1-RNAi, or both transgenes versus driver-only controls. Flies were tested at 10 (C) and 16 (D) d of age. n > 100 per genotype. **, P < 0.01; ***, P < 0.005; Mann-Whitney test. (E and F) Transcript levels of caz (E) and Xrp1 (F) in heads of adult female flies 3 d after induction of ubiquitous FUS-R518K expression (tub-GAL4) versus driver-only controls. n = 9–11. ***, P < 0.0005; two-tailed unpaired t test. (G and H) Caz (G) and Xrp1 (H) transcript levels in the CNS of third instar female larvae heterozygous for caz2 or cazKO versus WT controls. n = 11–13. ***, P < 0.001; one-way ANOVA. All graphs display mean ± SEM.