Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery
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Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi · Feb 2018
[The experimental study on endoplasmic reticulum stress-participated outer hair cell apoptosis in cadherin 23 gene mutant mice].
Objective: To test the mechanism and upstream pathway of outer hair cell apoptosis in Cadherin 23 (Cdh23) gene mutant mice. Method: The mutant Cdh23(erl/erl)(erl) mice were collected as the study group, while the C57BL/6J (B6) mice were chosen as the control group. A total of 70 mice per group were used in this study. ⋯ On the contrary, the CDH23(erl) protein was found to be localized from the top to the nuclei of the OHC in erl mice. Portions of the CDH23(erl) proteins failed to reach the top of the hair bundles and remained in the OHC cytoplasm. Conclusion: As the downstream response of the Cdh23 gene mutation, portions of the mutant CDH23(erl) protein was accumulated in ER lumen resulting in the increase of ER loading and ultimately triggered ER stress and hair cell apoptosis in erl mouse cochleae.