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Caption Cochlear hair cell abnormalities in rda/rda and Elmod1rda-2J/ Elmod1rda-2J (rda-2J) mice. Examination of the apical surfaces of cochlear hair cells by scanning electron microscopy showed normal bundle morphology in both inner hair cells (IHC) and outer hair cells (OHC) of rda/rda mutant mice at P0 (A). At P7 the stereocilia adjacent to the kinocilium (marked by arrow in H) had degenerated in some of the OHC bundles (marked by asterisks in B, D) of rda/rda mice, compared with the fully intact bundles of wild-type (+/+) controls (C). IHC bundles of rda/rda mice retained a normal appearance at P10 (D, K), but by P15 (F, L) all IHCs of mutant mice exhibited stereocilia elongations and fusions as compared with the normal IHC bundle morphology of age-matched controls (E). IHCs at P35 illustrate the striking degree of stereocilia elongations and fusions seen in rda/rda mutants (N) compared with age-matched controls (M). In contrast to IHCs, the OHC stereocilia of rda/rda mice did not elongate or fuse, but they did degenerate over time. By P15, nearly all OHCs had lost stereocilia at the bundle peak and a few (marked by asterisks) had lost the entire bundle (F, I), as compared with the intact OHC bundles of control mice (J). The IHC and OHC bundle abnormalities of Elmod1rda-2J/ Elmod1rda-2J mutant mice (G) were very similar to those of rda/rda mice (F). Scale bars: A-G, 10 microns; H-N, 5 microns. All hair cells shown in the figure are from the middle region of the cochlea, but similar bundle abnormalities were seen in other regions (not shown), consistent with the absence of an ABR at all test frequencies.
Copyright This image is from Johnson KR, PLoS One 2012;7(4):e36074, and is displayed under the terms of the Creative Commons Attribution 4.0 International License. J:187273
Symbol Name
Elmod1rda-2J ELMO/CED-12 domain containing 1; roundabout 2 Jackson
rda roundabout
Allelic Composition Genetic Background
rda/rda C57BL/6J-rda/JKjn
Elmod1rda-2J/Elmod1rda-2J B6.Cg-Elmod1rda-2J/JKjn

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