Progress in Research on Hereditary Non-syndromic Hearing Loss Associated with Mitochondrial Dysfunctions
ZHAO Jingjing
WANG Qixuan
LIN Xin
LI Gen
SONG Lei
WU Hao
Abstract：Mitochondria possess genetic materials independent of the nuclear genes.Mitochondrial dysfunctions cause a variety of diseases including hereditary non-syndromic hearing loss.Auditory cells that operate on high energy expenditure from oxidative phosphorylation require high level of mitochondrial activities.Mitochondrial gene mutations that affect the functions of important proteins result in abnormal oxidative phosphorylation processes,which in turn trig-ger downstream activation of ROS-AMPK-E2F1 pathway and cause damage and apoptosis of inner ear cells.This paper reviews a few recently established animal models used in studies of human mitochondrial gene mutations associated with hereditary non-syndromic hearing loss.The review summarizes underlying cellular and molecular mechanisms in several types of hearing loss due to mitochondrial dysfunctions.Clinical relevance and future treatment regimens are al-so discussed.
Keywords：Mitochondrial DysfunctionHereditary Non-syndromic Hearing LossAnimal ModelsROS-AMPK-E2F1 Pathway
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 136-140 )