Figure 4.

Dual-AAV8(Y733F)-MYO7A vectors improve vestibular stereocilium organization and circling behavior in shaker-1 mice

(A) Representative confocal images of stereocilium bundles of vestibular hair cells from mouse utricles stained by phalloidin to visualize F-actin. The shaker-1 vestibular hair cells have highly disorganized stereocilium bundles compared with wild-type hair cells. Shaker-1 mice treated with dual-AAV8(Y733F)-MYO7A vectors showed significant improvement in vestibular stereocilium organization compared with untreated shaker-1 vestibular hair cells. The scale bar represents 10 μm. (B) Representative track plots from a wild-type mouse, an untreated shaker-1 mouse, and a shaker-1 mouse treated with dual-AAV8(Y733F)-MYO7A vectors. Dual-AAV8(Y733F)-MYO7A vectors decreased circling behavior in treated shaker-1 mice. (C) Quantification of circling behavior was performed at ∼P30. Shaker-1 mice that were treated with dual-AAV8(Y733F)-MYO7A showed significant reduction in circling compared with untreated shaker-1 mice. Error bars represent standard errors. ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001, ∗∗∗∗p < 0.0001.