Impaired AIF-CHCHD4 interaction and mitochondrial calcium overload contribute to auditory neuropathy spectrum disorder in patient-iPSC-derived neurons with AIFM1 variant

Impaired AIF-CHCHD4 interaction and mitochondrial calcium overload contribute to auditory neuropathy spectrum disorder in patient-iPSC-derived neurons with AIFM1 variant

Abstract Auditory neuropathy spectrum disorder (ANSD) is a hearing impairment caused by dysfunction of inner hair cells, ribbon synapses, spiral ganglion neurons and/or the auditory nerve itself. Approximately 1/7000 newborns have abnormal auditory nerve function, accounting for 10%-14% of cases of...

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Bibliographic Details
Main Authors: Yue Qiu, Hongyang Wang, Mingjie Fan, Huaye Pan, Jing Guan, Yangwei Jiang, Zexiao Jia, Kaiwen Wu, Hui Zhou, Qianqian Zhuang, Zhaoying Lei, Xue Ding, Huajian Cai, Yufei Dong, Lei Yan, Aifu Lin, Yong Fu, Dong Zhang, Qingfeng Yan, Qiuju Wang
Format: Article
Language:English
Published: Nature Publishing Group 2023-06-01
Series:Cell Death and Disease
Online Access:https://doi.org/10.1038/s41419-023-05899-6

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