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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
Published on: February 21, 2016
Ultrastructural pathological changes in the cochlear cells of connexin 26 conditional knockout mice
Lan Lin1, Yun-Feng Wang, Shu-Yi Wang
1Department of Pathology, Eye and ENT Hospital of Fudan University, Shanghai 200031, P.R. China.
Molecular Medicine Reports
|August 7, 2013
Summary
Connexin 26 (Cx26) gene mutations cause hereditary deafness. Cx26 knockout mice show abnormal cochlear ultrastructure and cell death, suggesting microglia-like cells may contribute to degeneration.
Area of Science:
- Genetics
- Otolaryngology
- Cell Biology
Background:
- Mutations in the connexin 26 (Cx26) gene are the primary cause of non-syndromic hereditary hearing loss.
- The precise mechanisms driving deafness associated with Cx26 mutations are not fully understood.
Purpose of the Study:
- To investigate the ultrastructural changes and cellular markers in the cochlear sensory epithelium of Cx26 conditional knockout mice.
- To elucidate the underlying mechanisms of deafness resulting from Cx26 null mutations.
Main Methods:
- Examined cochlear specimens from Cx26 conditional knockout (cCx26ko) mice and wild-type controls.
- Utilized transmission electron microscopy for ultrastructural analysis.
- Employed antibodies for pillar cell (P75), supporting cell (prox1), and hair cell (myosin 6, phalloidin) markers.
Main Results:
- Early stages showed no significant changes in hair and supporting cell markers.
- The tunnel of the organ of Corti and Nuel's space failed to develop before hearing onset in cCx26ko mice.
- Cell death initiated around postnatal day 10 (P10), with microglia-like cells appearing in surviving Hensen cells post-P180.
Conclusions:
- Cx26 deficiency leads to abnormal ultrastructural morphology in the cochlear sensory epithelium.
- Microglia-like cells may play a role in the degenerative processes observed in Cx26-deficient cochleas.
