Related Experiment Video For Cdh23
Updated: Mar 27, 2026
Dissection of Adult Mouse Utricle and Adenovirus-mediated Supporting-cell Infection
Published on: March 28, 2012
Tauroursodeoxycholic acid prevents hearing loss and hair cell death in Cdh23(erl/erl) mice
1Department of Otorhinolaryngology-Head & Neck Surgery, Second Affiliated Hospital, Xi'an Jiaotong University School of Medicine, 157 Xiwu Road, Xi'an 710014, Shaanxi, PR China; Department of Otolaryngology-Head & Neck Surgery, Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
Abstract:
Sensorineural hearing loss has long been the subject of experimental and clinical research for many years. The recently identified novel mutation of the Cadherin23 (Cdh23) gene, Cdh23(erl/erl), was proven to be a mouse model of human autosomal recessive nonsyndromic deafness (DFNB12). Tauroursodeoxycholic acid (TUDCA), a taurine-conjugated bile acid, has been used in experimental research and clinical applications related to liver disease, diabetes, neurodegenerative diseases, and other diseases associated with apoptosis. Because hair cell apoptosis was implied to be the cellular mechanism leading to hearing loss in Cdh23(erl/erl) mice (erl mice), this study investigated TUDCA's otoprotective effects in erl mice: preventing hearing impairment and protecting against hair cell death. Our results showed that systemic treatment with TUDCA significantly alleviated hearing loss and suppressed hair cell death in erl mice. Additionally, TUDCA inhibited apoptotic genes and caspase-3 activation in erl mouse cochleae. The data suggest that TUDCA could be a potential therapeutic agent for human DFNB12.
