Volume 17, No 3, Mar 2007
ISSN: 1001-0602
EISSN: 1748-7838 2018
impact factor 17.848*
(Clarivate Analytics, 2019)
Volume 17 Issue 3, March 2007: 227-239
REVIEWS
Mitochondrial rRNA and tRNA and hearing function
Guangqian Xing1, Zhibin Chen1, Xin Cao2
1Department of Otolaryngology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China; 2Department
of Biotechnology, Nanjing Medical University, Nanjing 210029, China
Correspondence: Guangqian Xing(xing-gq@163.com)
The human ear is a delicate sensory apparatus of hearing for normal communication, and its proper functioning is highly dependent on mitochondrial oxidative phosphorylation. The first mitochondrial point mutation for nonsyndromic and aminoglycoside-induced hearing loss was identified in 1993. Since then a number of inherited mitochondrial mutations have been implicated in hearing loss. Most of the molecular defects responsible for mitochondrial disorder-associated hearing loss are mutations in the 12S rRNA gene and tRNA genes. In this review, after a short description of normal hearing mechanisms and mitochondrial genetics, we outline the recent advances that have been made in the identification of deafness-associated mitochondrial mutations, and discuss how mitochondrial dysfunction contributes to hearing loss.
Cell Research (2007) 17: 227-239. doi: 10.1038/sj.cr.7310124; published online 2 January 2007
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