Phenotype Image Detail

Image

Caption

Scanning electron microscopy of the organ of Corti in Tmprss3^m1Ingm/Tmprss3^m1Ingm (Tmprss3^Y260X, A-E) and wild-type (WT) mice (F-J). A, 7 days; basal cochlear region. The stereociliary bundles are already well formed on both the row of inner hair cells (IHCs) (I) and the three rows of outer hair cells (OHCs) (O). Each bundle is associated with a thick kinocilium (see the inset). The distance between IHCs and the first OHC row is, however, very small because the heads of pillar cells (p) are not yet fully developed. The supporting cells are covered with long, densely packed microvilli. B, 11 days (basal cochlear region). The surface of the organ of Corti has a mature aspect. The stereociliary bundles are typically organized in one row for IHCs and in three rows for OHCs. Both types of hair cells are separated by the heads of pillar cells. Consistent with this stage of cochlear development, the thick kinocilium has already completely regressed on OHCs. In the inset, an OHC shows an additional thin and very long kinocilium (arrow) near the footprint of the preexisting thick kinocilium (arrowhead). Behind the last row of OHCs, the footprints of the transient marginal pillars are visible (asterisks). C, 12 days; basal cochlear region. Both IHCs and OHCs have disappeared. The supporting cells have invaded the area of hair cells to form a scar, but in some places (right half of the picture), the wound is not completely healed. The inset shows an OHC partially extruded from the epithelium. D, 13 days; apical cochlear region. Both types of hair cells are missing throughout the cochlea except for some remaining hair cells in the apical extremity. E, 2 months; middle cochlear region. The hair cells are missing, and the scarring process is achieved all along the cochleae. F-J allow comparison of the organs of Corti in WT mice with those of homozygous mutant mice, at the same ages and at the same levels of the cochlear spiral. In the WT mice, the organs of Corti have a normal appearance and show all hair cells, whatever the conditions. F, 7 days; basal cochlear region. G, 11 days; basal cochlear region. H, 12 days; basal cochlear region. I, 13 days; apical extremity. J, 2 months; middle cochlear region. The organ of Corti has an adult-like aspect. The density of microvilli is low. Note the absence of marginal pillar footprints. Scale bars (A-H), 10 um; insets, 1 um.

Copyright

This image is from Fasquelle L, J Biol Chem 2011 May 13;286(19):17383-97 and is displayed with the permission of the American Society for Biochemistry and Molecular Biology who owns the Copyright. Full text from JBC. J:172676

Associated Alleles

Symbol	Name
Tmprss3^m1Ingm	transmembrane protease, serine 3; mutation 1, Ingenium Pharmaceuticals

Associated Genotypes

Allelic Composition	Genetic Background
Tmprss3^m1Ingm/Tmprss3^m1Ingm	C3HeB/FeJ-Tmprss3^m1Ingm