Figure 7.

USH2 proteins are mislocalized in Pdzd7^−/− cochlear hair cells. (A) GPR98 (red) was present at ankle links of hair bundles (phalloidin, green) in Pdzd7^+/+ (WT) cochlear hair cells. In Pdzd7^−/− mice (KO), only a small amount of GPR98 remained at ankle links. Most GPR98 moved upward toward the tip or along the stereocilia in IHC and OHC bundles. (B) WHRN (red) was present at the tip and ankle links of hair bundles (green, phalloidin) in Pdzd7^+/+ cochlear hair cells. In Pdzd7^−/− cochleas, the WHRN signals at the hair bundle tip stayed unchanged, while the signals at ankle links largely disappeared. (C) USH2A (red) was present at ankle links of the hair bundle (green, phalloidin) in Pdzd7^+/+ cochlear hair cells. In about half of Pdzd7^−/− OHCs, partial USH2A signal was mislocalized to the tip of the hair bundle, while the rest was still present at ankle links. In Pdzd7^−/− IHCs, USH2A signals were significantly reduced at ankle links. Arrowheads point to the base of hair bundles. Scale bars, 1 µm.