Nobutaka Ohgami, Haruka Tamura, Kyoko Ohgami, Machiko Iida, Ichiro Yajima, Mayuko Y Kumasaka, Yuji Goto, Michihiko Sone, Tsutomu Nakashima, Masashi Kato
Units of Environmental Health Sciences, Department of Biomedical Sciences, College of Life and Health Sciences,Chubu University, Kasugai, Aichi, Japan.
International journal of clinical and experimental pathology 2012About 120 million people worldwide suffer from congenital (early-onset) hearing loss. Thirty percent of them have syndromic hearing loss and the remaining 70% have non-syndromic hearing loss. In addition, a large number of elderly people worldwide suffer from age-related (late-onset) hearing loss. c-Ret and c-RET have been shown to be essential for the development and maintenance of neurons including the enteric nervous system (ENS) in mice and humans. Impairments of endothelin receptor B (EDNRB) and SOX10 have been shown to cause a significantly increased risk of dominant sensorineural deafness in Hirschsprung disease (HSCR) patients. We have recently shown that impairments of tyrosine 1062 (Y1062) phosphorylation in c-Ret causes syndromic congenital deafness in mice and humans and non-syndromic age-related hearing loss with neurodegeneration of spiral ganglion neurons (SGNs) in mice. This review focuses on the pathogenesis of hearing loss caused by impairments of c-Ret.
Nobutaka Ohgami, Haruka Tamura, Kyoko Ohgami, Machiko Iida, Ichiro Yajima, Mayuko Y Kumasaka, Yuji Goto, Michihiko Sone, Tsutomu Nakashima, Masashi Kato. c-Ret-mediated hearing losses. International journal of clinical and experimental pathology. 2012;5(1):23-8
PMID: 22295143
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