title: c-Ret-mediated hearing losses.
abstract: About 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.
authors.affiliation: Units of Environmental Health Sciences, Department of Biomedical Sciences, College of Life and Health Sciences,Chubu University, Kasugai, Aichi, Japan.
authors.forename: Nobutaka
authors.initials: N
authors.lastname: Ohgami
authors.forename: Haruka
authors.initials: H
authors.lastname: Tamura
authors.forename: Kyoko
authors.initials: K
authors.lastname: Ohgami
authors.forename: Machiko
authors.initials: M
authors.lastname: Iida
authors.forename: Ichiro
authors.initials: I
authors.lastname: Yajima
authors.forename: Mayuko Y
authors.initials: MY
authors.lastname: Kumasaka
authors.forename: Yuji
authors.initials: Y
authors.lastname: Goto
authors.forename: Michihiko
authors.initials: M
authors.lastname: Sone
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Nakashima
authors.forename: Masashi
authors.initials: M
authors.lastname: Kato
mesh_terms: D000818:Animals; D034381:Hearing Loss / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D051379:Mice; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics* / Q000378:metabolism
chemical_list: D051096:Proto-Oncogene Proteins c-ret