Fig. 5. The OPTN-E50K point mutation mediates the degradation of TDP-43 via autophagy.
A Western blot analysis of TDP-43 protein expression in the retinal cytoplasm and nucleus. β-tubulin and histone h3 were used as loading controls to validate the fractionation of cellular extracts. B, C The protein expression of TDP-43 was significantly greater in the cytoplasm and was significantly lower in the nucleus in the retinas of E50K mutant mice than in those of WT mice. n = 3. D Fluorescence analysis of the localisation of TDP-43 and optineurin in retinas of WT and E50K mutant mice. TDP-43-positive granules (red) were more colocalized with optineurin signals (green) in E50K mutant mice, and E and F both the values of Pearson’s correlation and Manders’ overlap coefficient between OPTN and TDP-43 were significantly higher in E50K mutant mice than in WT mice. n = 10/14. G Western blot analysis of immunoprecipitation assays of TDP-43 or OPTN in retinas. *P < 0.05; **P < 0.01.