Fig. 5

Exogenous Tmc1 or Tmc2 restores vestibular function in Tmc1^∆/∆;Tmc2^∆/∆ mice. a Rotational vestibular ocular reflexes (RVOR) to sinusoidal head rotations with representative eye velocity responses to 1 Hz head rotation for a wild-type mouse (black), a Tmc1^∆/∆;Tmc2^∆/∆ mouse (red), an sAAV-Tmc2-injected mouse (green). b Mean ± S.D. RVOR gains and c phases for WT group (n = 5, black), uninjected Tmc mutant mice (n = 4, red), and sAAV-Tmc2-treated group (n = 5, green). d Linear vestibular ocular reflexes (LVORs) to sinusoidal head translations with representative eye position measurements to 2 Hz head translations for a wild-type mouse (black), a Tmc1^∆/∆;Tmc2^∆/∆ mouse (red), an sAAV-Tmc2-injected mouse (green). e Mean ± S.D. gains and f phases of the LVORs are shown for uninjected Tmc mutant group (n = 4, red), the treated group (n = 5, green) and the WT group (n = 5, black). g Eye velocity response curves due to a high acceleration and high frequency step rotation. h Mean gains ± S.D. for eye movements during the first 100 ms, as shown in g. For a–g, positive head movements is for rightward head rotation and positive eye movement is for eye movement toward the left ear. Eye movements in injected and wild-type groups were compensatory to head rotation. Stars indicate statistical significance (t-test). i Mean ± S.D. number of full body rotations observed during 5 min in a 42-cm-wide arena in 6-week-old control Tmc1^+/+;Tmc1^+/+ mice, Tmc1^∆/∆; Tmc2^∆/∆ mice, and Tmc1^∆/∆; Tmc2^∆/∆ mice injected at either P1, P14 or P30 with sAAV-Tmc1 or sAAV-Tmc2. Significant recovery P < 0.001 was observed between the uninjected and injected mice. Number of mice test for each of eight conditions were: 6, 4, 6, 8, 5, 7, 7, 7, respectively. Statistical analysis by one-way ANOVA. See Supplementary Movies 1-3. j Mean ± S.D. time spend on rotarod device recorded at 6 weeks of age in control Tmc1^+/+;Tmc1^+/+ mice, Tmc1^∆/∆; Tmc2^∆/∆ mice, and Tmc1^∆/∆; Tmc2^∆/∆ mice injected at either P1, P14 or P30 with sAAV-Tmc1 or sAAV-Tmc2. Number of animals test for each of eight conditions were: 10, 5, 7, 10, 6, 7, 10, 10, respectively