期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 23, 期 20, 页码 -出版社
MDPI
DOI: 10.3390/ijms232012393
关键词
inner ear; cochlear hair cells; RNA-binding proteins; post-transcriptional regulation; sensorineural hearing loss; zebrafish neuromasts; regeneration
资金
- National Natural Science Foundation of China [32070813]
- French Muscular Dystrophy Association (AFM-Telethon) [23545]
- Centre National de la Recherche Scientifique (CNRS)
- Sorbonne University
- National Natural Science Foundation of China
RNA-binding proteins (RBPs) have important post-transcriptional regulatory roles in inner ear development and hair cell function, and defects in their function can lead to deafness. Understanding the events regulated by RBPs can help in defining therapeutic strategies for hearing loss treatment.
RNA-binding proteins (RBPs) regulate gene expression at the post-transcriptional level. They play major roles in the tissue- and stage-specific expression of protein isoforms as well as in the maintenance of protein homeostasis. The inner ear is a bi-functional organ, with the cochlea and the vestibular system required for hearing and for maintaining balance, respectively. It is relatively well documented that transcription factors and signaling pathways are critically involved in the formation of inner ear structures and in the development of hair cells. Accumulating evidence highlights emerging functions of RBPs in the post-transcriptional regulation of inner ear development and hair cell function. Importantly, mutations of splicing factors of the RBP family and defective alternative splicing, which result in inappropriate expression of protein isoforms, lead to deafness in both animal models and humans. Because RBPs are critical regulators of cell proliferation and differentiation, they present the potential to promote hair cell regeneration following noise- or ototoxin-induced damage through mitotic and non-mitotic mechanisms. Therefore, deciphering RBP-regulated events during inner ear development and hair cell regeneration can help define therapeutic strategies for treatment of hearing loss. In this review, we outline our evolving understanding of the implications of RBPs in hair cell formation and hearing disease with the aim of promoting future research in this field.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据