Ablation of Whirlin Long Isoform Disrupts the USH2 Protein Complex and Causes Vision and Hearing Loss
Figure 5
Whirlin and usherin are colocalized in photoreceptors.
(A) Whirlin (green) and usherin (red) were colocalized at the PMC in mouse photoreceptors. The bottom panels are the enlarged view of the region marked by the frame in the merged image on the top. (B) The signals of usherin (red, top) and whirlin (green, bottom) were mislocalized from the PMC in whirlin knockout (whirlin−/−), whirler mutant (whirlinwi/wi) and Ush2a knockout (Ush2a−/−) mice (see results section for details). OS, outer segments; CC, connecting cilia; IS, inner segments. Scale bars (A,B), 5 µm. (C) The amount of usherin was reduced by about 80% in the retina of whirlin knockout mice as analyzed by immunoblotting. (D) The amount of whirlin was reduced by about 50% in the retina of Ush2a knockout mice as analyzed by immunoblotting. The images on the top of the bar charts in (C,D) are the representative western blots of usherin and whirlin, respectively. The signals of α-tubulin were used as a loading control. Error bars in (C,D) represent the standard error of the mean. The numbers in the bottom of each bar (C,D) are the numbers of animals analyzed.