Nuclear PABPN1 aggregates in the OPMD <i>Drosophila</i> model.

<p>A) Genetic rescue of wing position phenotypes with <i>twin</i><sup><i>KG00877</i></sup> hererozygous mutant. Percentage of wing posture defects in the presence or absence of <i>twin</i><sup><i>KG00877</i></sup>/+, scored at day 6. OPMD flies were <i>w</i><sup><i>1118</i></sup><i>; UAS-PABPN1-17ala/+; Mhc-Gal4/+</i> raised at 18°C. *** <i>p</i>-value <0.0001, using the χ<sup>2</sup> test. B) Immunostaining of indirect flight muscles from OPMD (<i>w</i><sup><i>1118</i></sup><i>; UAS-PABPN1-17ala/+; Mhc-Gal4/+)</i> and OPMD; <i>twin</i><sup><i>KG00877</i></sup><i>/+</i> (<i>w</i><sup><i>1118</i></sup><i>; UAS-PABPN1-17ala/+; Mhc-Gal4/twin</i><sup><i>KG00877</i></sup>) adult flies at day 6 with anti-PABPN1 (green), showing nuclear aggregates. DNA was visualized with DAPI (blue). Nuclei are outlined with a dotted line. C) Quantification of PABPN1 nuclear aggregates. (Top) Percentages of nuclei with a nuclear PABPN1 aggregate in OPMD and OPMD; <i>twin</i><sup><i>KG00877</i></sup><i>/+</i> indirect flight muscles (genotypes as in B) *** <i>p</i>-value <0.0001, using the χ<sup>2</sup> test. (Bottom) Quantification of nuclear aggregate areas. Each nuclear aggregate was delimited in a focal plan and the surface was calculated using ImageJ. Mean values of the areas with standard errors of the mean are indicated in arbitrary units Distribution of cross-sectional areas is shown as box plots (right), the median is indicated as an horizontal line within the box. ns: not significant, using the Student’s t-Test. Thoracic muscles were stained with anti-PABPN1 and DAPI and nuclear aggregates were visualized and scored using both staining. Quantification was from three independent experiments.</p>