线粒体耳聋的研究进展

Research progress of mitochondrial deafness

摘要:

线粒体耳聋是线粒体功能障碍引起的听力损失，主要由线粒体DNA(mtDNA)突变引起，受核基因调控，并和环境因素等相互作用。线粒体功能障碍不仅会引起遗传性综合征性耳聋和非综合征性耳聋，而且参与年龄相关性耳聋的发病。近年来线粒体耳聋越来越受到人们关注，各个方面的研究也取得了一定的进展。因此，本文从线粒体耳聋的研究模型、致病机制、研究方法以及干预策略等方面的进展进行综述，旨在为将来该病的进一步研究提供参考。

Abstract:

Mitochondrial deafness is a hearing loss of mitochondrial dysfunction, which is mainly caused by mitochondrial DNA (mtDNA) mutations. Mitochondrial deafness is regulated by nuclear genes and affected by the environmental factors. Mitochondrial dysfunction is associated with not only hereditary syndromic and nonsyndromic deafness,but also with age-related deafness. In recent years, mitochondrial deafness has attracted more and more attention. Some progress has been made in the study of mitochondrial deafness. Therefore, this paper reviews the research models, pathogenic mechanisms, research methods, and intervention strategies of mitochondrial deafness.The purpose of the paper is to provide reference for the further study of the disease in the future.