Phenotype Image Detail

Image

Caption

Transmission electron microscopy of the organ of Corti in Tmprss3^m1Ingm/Tmprss3^m1Ingm mice. A-C, 11 days. A, upper basal region of the cochlea. The inner hair cells (IHCs) (I) and outer hair cells (OHCs) (O) are present and have a normal appearance. Their stereocilia (arrows) are well erected, and tip links are distinguishable (arrowhead in the upper inset). The tunnel of Corti (tC) and the spaces of Nuel around the OHCs are well opened. Numerous myelinated nerve fibers (lower inset) are present in the spiral lamina. Note the piece of a marginal pillar attached to the distal part of the tectorial membrane (asterisk). D, Deiters cells; ip, inner pillar cell; ep, external pillar cell; tm, tectorial membrane. B, basal pole of an IHC (I) contacted by several (radial) afferent endings (a). A synaptic ribbon (arrow) is present within the IHC, facing an afferent. C, basal pole of an OHC in contact with two (spiral) afferent endings and one larger efferent terminal (e). The arrows point to a postsynaptic cistern within the OHC. D-F, 12 days; middle cochlear region. D, the hair cells have disappeared from the epithelium and are replaced by extensions of Deiters cells in the OHC area and by inner supporting cells, including phalangeal cells (pc), in the IHC area. The external and inner pillar cells are collapsed. The tectorial membrane (not seen on the picture) has a normal appearance. E, in the IHC region, corpses of hair cells (asterisks) have been extruded from the epithelium into the endolymphatic space (border cells (bc)). The arrows point to cellular debris within the cytoplasm of a phalangeal cell. F, enlargement of a damaged IHC remnant (asterisk) seen in D. The IHC remnant is wrapped by a phalangeal cell and shows dark mitochondria and a dense cytoplasm. Scale bars, 10 um (A and D) and 1 um (B, C, E, F, and lower inset in A), and 0.1 um (upper inset in A).

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