• Neuroreport · Feb 2019

    Chemical chaperone 4-phenylbutyrate prevents hearing loss and cochlear hair cell death in Cdh23erl/erl mutant mice.

    • Bo Li, Tihua Zheng, Caifang Yan, Wenjun Wang, Jinjin Zhang, Luping Zhang, Juan Hu, Li Zhang, Yuzhu Wan, Minyan Zhang, and Qingyin Zheng.
    • Transformative Otology and Neuroscience Center, Binzhou Medical University, Yantai, People's Republic of China.
    • Neuroreport. 2019 Feb 6; 30 (3): 145-150.

    AbstractWe previously developed Cdh23 mutant mice (erl mice) as a model of hearing loss for otoprotective drug evaluation and showed that the erl mutation leads to hearing loss related to endoplasmic reticulum (ER) stress-induced cochlear hair cell apoptosis. Small molecular chemical chaperones, 4-phenylbutyrate (4PBA), targeting ER stress exert a neuroprotective effect. To evaluate whether 4PBA exerts an otoprotective effect, we intraperitoneally injected erl mice with 4PBA daily from postnatal age day 7 up to 12 weeks. Our results showed that treatment with 4PBA significantly alleviated hearing loss and suppressed hair cell death in erl mice. In addition, ER stress-related proteins were downregulated by 4PBA treatment. Our study showed that 4PBA exerts an otoprotective effect, which provides the potential to repurpose the drug for otoprotection.

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