Article
Dysregulation of Serpinb6a-Gch1 axis contributes to DFNB91 deafness that is amendable to gene therapies.
Molecular therapy : the journal of the American Society of Gene Therapy - 6 May 2026
Cheng Cheng, Zhang Liyan, Lu Jie, Tan Fangzhi, Huang Yideng, Gao Song, Li Siyu, Qiu Yue, Hao Wenli, Zhou Yingyi, Lu Junze, Ji Xinya, Li Ao, Zhang Xinru, Fan Jinyi, Li He, Qian Xiaoyun, Qi Jieyu, Gao Xia, Chai Renjie
Abstract excerpt
Hereditary deafness accounts for 60% of congenital hearing loss, and more than 300 deafness genes have been identified. SERPINB6, a gene associated with recessive deafness, also named DFNB91, is linked to non-syndromic progressive hearing loss based on studies of humans and its mouse ortholog Serpinb6a knockout mice. However, the mechanism and biological therapy for SERPINB6 mutation-induced deafness remain...
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