C-Ret-mediated hearing losses

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

Nobutaka Ohgami, Haruka Tamura, Kyoko Ohgami, Machiko Iida, Ichiro Yajima, Mayuko Y. Kumasaka, Yuji Goto, Michihiko Sone, Tsutomu Nakashima, Masashi Kato

Research output: Contribution to journal › Review article › peer-review

3 Citations (Scopus)

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.

Original language	English
Pages (from-to)	23-28
Number of pages	6
Journal	International Journal of Clinical and Experimental Pathology
Volume	5
Issue number	1
Publication status	Published - 2012
Externally published	Yes

All Science Journal Classification (ASJC) codes

Pathology and Forensic Medicine
Histology

Cite this

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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.",

author = "Nobutaka Ohgami and Haruka Tamura and Kyoko Ohgami and Machiko Iida and Ichiro Yajima and Kumasaka, {Mayuko Y.} and Yuji Goto and Michihiko Sone and Tsutomu Nakashima and Masashi Kato",

year = "2012",

language = "English",

volume = "5",

pages = "23--28",

journal = "International Journal of Clinical and Experimental Pathology",

issn = "1936-2625",

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TY - JOUR

T1 - C-Ret-mediated hearing losses

AU - Ohgami, Nobutaka

AU - Tamura, Haruka

AU - Ohgami, Kyoko

AU - Iida, Machiko

AU - Yajima, Ichiro

AU - Kumasaka, Mayuko Y.

AU - Goto, Yuji

AU - Sone, Michihiko

AU - Nakashima, Tsutomu

AU - Kato, Masashi

PY - 2012

Y1 - 2012

N2 - 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.

AB - 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.

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M3 - Review article

C2 - 22295143

AN - SCOPUS:84856731691

SN - 1936-2625

VL - 5

SP - 23

EP - 28

JO - International Journal of Clinical and Experimental Pathology

JF - International Journal of Clinical and Experimental Pathology

IS - 1

ER -

C-Ret-mediated hearing losses

Abstract

All Science Journal Classification (ASJC) codes

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