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. Author manuscript; available in PMC: 2020 Apr 1.
Published in final edited form as: Hear Res. 2019 Feb 22;375:14–24. doi: 10.1016/j.heares.2019.02.007

Figure 4:

Figure 4:

Localization of whirlin isoforms in the wild-type and Whrn mutant inner ear and retina. (A) In the developing wild-type organ of Corti (e.g., during mouse P2–P12), FL-whirlin is localized to the stereociliary base of IHCs and OHCs. Additionally, IHC stereociliary tips contain FL- and C-whirlin, while OHC stereociliary tips contain only C-whirlin. (B) Localization of whirlin isoforms in wild-type VHCs is similar to that of developing IHCs as shown in A. (C) FL- and C-whirlin isoforms remain at the stereociliary tip of mature wild-type IHCs, but no whirlin isoforms are present in mature wild-type OHCs. (D) FL-whirlin and likely N-whirlin are localized to the PMC in wild-type photoreceptor cells. (E-G) C-whirlin is localized to the stereociliary tip of OHCs, IHCs, and VHCs in Whrnneo mice. This localization of C-whirlin in OHCs occurs only during development but not in adulthood. No whirlin isoforms are present at the stereociliary base in any Whrnneo hair cells at any time point. (H) No whirlin isoforms are detected in Whrnneo photoreceptors. (I-K) Whrnwi inner ear hair cells do not show any whirlin expression at any time point. (L) The truncated N-whirlin fragment in Whrnwi retinas localizes to the PMC. (M-N) 96% of IHCs and 33% of OHCs in Whrnwi+BAC mice show localization of C-whirlin to their stereociliary tips during development. (O) Similar to Whrnneo mouse, Whrntm1a and Whrntm1b mice show localization of only C-whirlin to their IHC and OHC stereociliary tips during development. In this figure, the short and thick stereocilia phenotypes in Whrnneo and Whrnwi hair cells are shown.