Ablation of Whirlin Long Isoform Disrupts the USH2 Protein Complex and Causes Vision and Hearing Loss (original) (raw)
Figure 10
Whirlin knockout mice have non-progressive hearing defects.
(A) DPOAE analysis demonstrates that whirlin knockout mice have profound hearing loss at all stimulus frequencies, as measured at either 2 or 9 months of age. (B) Scanning electron microscopy shows scattered loss of outer hair cells (arrows) in the basal turn and dysmorphology of the stereocilia bundles in all cochlear regions (e.g. open arrowheads) in whirlin knockout mice. (C) At high magnification, in whirlin knockout mice, the inner hair cell stereocilia appear normal (left column); the outer hair cells show patchy loss of stereocilia in the innermost (shortest) row of the hair bundle (arrowheads, middle column); the interstereocilia links of the outer hair cells appear normal (arrowheads, right column). OH, outer hair cells; IH, inner hair cells. Scale bars, 5 µm (B) and 1 µm (C).