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A novel splice site mutation of myosin VI in mice leads to stereociliary fusion caused by disruption of actin networks in the apical region of inner ear hair cells

Fig 1

Homozygous ksv mutation in mice causes balance defects and stereociliary degeneration of vestibular hair cells.

A and B. Representative open-field pathway traces (white curved line) at 1 month of age in wild-type (+/+) (A) and ksv/ksv (B) mice. C and D. SEM images of stereocilia from the utricles in the +/+ (C) and ksv/ksv (D) mice at P14. Highly magnified images of stereociliary bases (dotted boxes) in C and D are shown in each bottom panel (C’ and D’). Arrows and arrowhead indicate a bulged basal region and a loss of tapered region, respectively, in the stereocilia of ksv/ksv mice. Scale bars = 5 μm (C and D), and 2 μm (C’ and D’).

Fig 1

doi: https://doi.org/10.1371/journal.pone.0183477.g001