Kefan Tao1 · Yanjun Zong1 · Xiaozhou Liu1 · Xinyu Shi1 · Zhengdong Zhao1 · Yu Sun1,2,31 Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China
2 Institute of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China
3 Hubei Province Clinic Research Center for Deafness and Vertigo, Wuhan 430022, China
Abstract
Hearing loss is one of the most prevalent sensory disorders affecting the human nervous system. Liquid–liquid phase separation (LLPS) is a physiological process that facilitates the reversible and dynamic assembly of biomolecular condensates. Increasing evidence suggests that LLPS plays a significant role in the pathogenesis of hereditary hearing loss. Nevertheless, there is a conspicuous lack of systematic investigations exploring the impact of LLPS abnormalities on the etiology of hereditary hearing loss. In this review, we examine the mechanisms by which dysfunctions in LLPS contribute to hereditary hearing loss, specifically focusing on its effects on mechanoelectrical transduction in hair bundles, transcriptional regulation, post-transcriptional modifications, the actin cytoskeleton, ion homeostasis within the inner ear, and energy and redox homeostasis. Furthermore, we evaluate the considerable potential of targeting LLPS as a therapeutic approach for hearing loss and propose innovative perspectives on LLPS that may guide future research initiatives in the field of auditory disorders.
Keywords
Liquid–liquid phase separation; Hereditary hearing loss; Usher syndrome; Ankle link complex; Posttranscriptional modifcations
