Mitochondrial rRNA and tRNA and hearing function.
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.
Xing
Guangqian
G
Department of Otolaryngology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China. xing-gq@163.com
Chen
Zhibin
Z
Cao
Xin
X
D000617:Aminoglycosides / Q000009:adverse effects; D000900:Anti-Bacterial Agents / Q000009:adverse effects; D006309:Hearing / Q000187:drug effects / Q000235:genetics / Q000502:physiology*; D034381:Hearing Loss / Q000235:genetics; D006801:Humans; D008928:Mitochondria / Q000235:genetics; D008954:Models, Biological; D009154:Mutation; D012313:RNA / Q000235:genetics / Q000502:physiology*; D000077278:RNA, Mitochondrial; D012335:RNA, Ribosomal / Q000235:genetics / Q000502:physiology*; D012343:RNA, Transfer / Q000235:genetics / Q000502:physiology*; D013577:Syndrome
D000617:Aminoglycosides; D000900:Anti-Bacterial Agents; D000077278:RNA, Mitochondrial; D012335:RNA, Ribosomal; D012313:RNA; D012343:RNA, Transfer