Fig. 5.

In utero downregulation of Gipc1 leads to PCP phenotype and HC maturation defects, including hair bundle integrity. (A,A′) Surface view of a mouse cochlea electroporated at E11.5 with sh-GFP (green) and labeled seven days later with phalloidin (red). HC development was not affected. (B-E′) The expression of shGIPC1b-GFP (green) leads to a PCP phenotype in HCs (B″). We observed a reduction of the apical surface area of the HC, and reduced pericuticular MyoVI expression (B′,C′,D′, green stars), and an impairment in hair bundle orientation and integrity (C′,D′). When downregulation of Gipc1 did not completely disrupt the HC-SC junction, Vangl2 expression is present (C″, green arrow), but when a strong HC phenotype is observed, there is a disruption of the HC-SC junction (E,E′). (F) Pie charts illustrating the variation in distribution of the HC phenotypes. Scale bar: 8 μm.