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. 2004 Aug 16;166(4):559–570. doi: 10.1083/jcb.200402007

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Copyright © 2004, The Rockefeller University Press

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Figure 2. — Deafness of Rdx ^−/− mice. (A) Loss of Preyer's reflex in Rdx ^−/− mice. Time-lapse photography captures Preyer's reflex in Rdx ^+/+ and Rdx ^+/− mice, head movements (arrows), but not in Rdx ^−/− mice. Two successive frames after a loud handclap (1-s interval) were superimposed. These 10-wk-old mice show similar growth rates. (B) ABR to stimuli of 70-dB SPL (20 kHz) in two sets of Rdx ^+/− intercross littermates (left, 5-wk-old; right 10-wk-old). Among 12 littermates in total, three show no ABR, and were afterwards genotyped as Rdx ^−/− mice (blue squares). (C) Hearing thresholds of 10-wk-old Rdx ^+/+, Rdx ^+/−, and Rdx ^−/− mice at various sound frequencies. Rdx ^+/+ and Rdx ^+/− mice show normal hearing thresholds (10–50 dB SPL), whereas Rdx ^−/− mice show profound deafness (hearing threshold, >70–90 dB SPL). (D) Toluidine blue–stained Epon semi-thin sections of the cochlea. No gross morphological difference is observed in the organ of Corti (double-headed arrows) including hair cells (single-headed arrows) between Rdx ^+/+ and Rdx ^−/− mice. Bars, 50 μm (top panels); 10 μm (bottom panels). (E) Western blot analysis of isolated cochleae of the Rdx ^+/+ , Rdx ^+/−, and Rdx ^−/− mice with anti-ERM pAb (TK89) that recognizes ezrin (E), radixin (R), and moesin (M) equally. In the Rdx ^−/− cochlea, radixin becomes undetectable without significant up-regulation of ezrin or moesin. Silver-stained bands of actin (Ac) in the same gels are present to show that an equal amount of cell lysate was applied in each lane.