Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing

Michiru Ida-Eto, Nobutaka Ohgami, Machiko Iida, Ichiro Yajima, Mayuko Y. Kumasaka, Kazutaka Takaiwa, Takashi Kimitsuki, Michihiko Sone, Tsutomu Nakashima, Toyonori Tsuzuki, Shizuo Komune, Masashi Yanagisawa, Masashi Kato

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Impairments of endothelin receptor B (Ednrb/EDNRB) cause the development of Waardenburg-Shah syndrome with congenital hearing loss, hypopigmentation, and megacolon disease in mice and humans. Hearing loss in Waardenburg- Shah syndrome has been thought to be caused by an Ednrbmediated congenital defect of melanocytes in the stria vascularis (SV) of inner ears. Here we show that Ednrb expressed in spiral ganglion neurons (SGNs) in inner ears is required for postnatal development of hearing in mice. Ednrb protein was expressed in SGNs from WT mice on postnatal day 19 (P19), whereas it was undetectable in SGNs from WT mice on P3. Correspondingly, Ednrb homozygously deleted mice (Ednrb -/- mice) with congenital hearing loss showed degeneration of SGNs on P19 but not on P3. The congenital hearing loss involving neurodegeneration of SGNs as well as megacolon disease in Ednrb -/- mice were markedly improved by introducing an Ednrb transgene under control of the dopamine β-hydroxylase promoter (Ednrb -/-;DBH-Ednrb mice) on P19. Neither defects of melanocytes nor hypopigmentation in the SV and skin in Ednrb -/- mice was rescued in the Ednrb -/-;DBH-Ednrb mice. Thus, the results of this study indicate a novel role of Ednrb expressed in SGNs distinct from that in melanocytes in the SV contributing partially to postnatal hearing development.

Original languageEnglish (US)
Pages (from-to)29621-29626
Number of pages6
JournalJournal of Biological Chemistry
Volume286
Issue number34
DOIs
StatePublished - Aug 26 2011

Fingerprint

Endothelin B Receptors
Spiral Ganglion
Audition
Hearing
Neurons
Stria Vascularis
Hearing Loss
Melanocytes
Megacolon
Hypopigmentation
Inner Ear
Defects
Mixed Function Oxygenases
Dopamine
Skin
Transgenes

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Molecular Biology

Cite this

Ida-Eto, M., Ohgami, N., Iida, M., Yajima, I., Kumasaka, M. Y., Takaiwa, K., ... Kato, M. (2011). Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing. Journal of Biological Chemistry, 286(34), 29621-29626. https://doi.org/10.1074/jbc.M111.236802

Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing. / Ida-Eto, Michiru; Ohgami, Nobutaka; Iida, Machiko; Yajima, Ichiro; Kumasaka, Mayuko Y.; Takaiwa, Kazutaka; Kimitsuki, Takashi; Sone, Michihiko; Nakashima, Tsutomu; Tsuzuki, Toyonori; Komune, Shizuo; Yanagisawa, Masashi; Kato, Masashi.

In: Journal of Biological Chemistry, Vol. 286, No. 34, 26.08.2011, p. 29621-29626.

Research output: Contribution to journalArticle

Ida-Eto, M, Ohgami, N, Iida, M, Yajima, I, Kumasaka, MY, Takaiwa, K, Kimitsuki, T, Sone, M, Nakashima, T, Tsuzuki, T, Komune, S, Yanagisawa, M & Kato, M 2011, 'Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing', Journal of Biological Chemistry, vol. 286, no. 34, pp. 29621-29626. https://doi.org/10.1074/jbc.M111.236802
Ida-Eto, Michiru ; Ohgami, Nobutaka ; Iida, Machiko ; Yajima, Ichiro ; Kumasaka, Mayuko Y. ; Takaiwa, Kazutaka ; Kimitsuki, Takashi ; Sone, Michihiko ; Nakashima, Tsutomu ; Tsuzuki, Toyonori ; Komune, Shizuo ; Yanagisawa, Masashi ; Kato, Masashi. / Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing. In: Journal of Biological Chemistry. 2011 ; Vol. 286, No. 34. pp. 29621-29626.
@article{c156bde8f9064ab5bbd79110d208916f,
title = "Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing",
abstract = "Impairments of endothelin receptor B (Ednrb/EDNRB) cause the development of Waardenburg-Shah syndrome with congenital hearing loss, hypopigmentation, and megacolon disease in mice and humans. Hearing loss in Waardenburg- Shah syndrome has been thought to be caused by an Ednrbmediated congenital defect of melanocytes in the stria vascularis (SV) of inner ears. Here we show that Ednrb expressed in spiral ganglion neurons (SGNs) in inner ears is required for postnatal development of hearing in mice. Ednrb protein was expressed in SGNs from WT mice on postnatal day 19 (P19), whereas it was undetectable in SGNs from WT mice on P3. Correspondingly, Ednrb homozygously deleted mice (Ednrb -/- mice) with congenital hearing loss showed degeneration of SGNs on P19 but not on P3. The congenital hearing loss involving neurodegeneration of SGNs as well as megacolon disease in Ednrb -/- mice were markedly improved by introducing an Ednrb transgene under control of the dopamine β-hydroxylase promoter (Ednrb -/-;DBH-Ednrb mice) on P19. Neither defects of melanocytes nor hypopigmentation in the SV and skin in Ednrb -/- mice was rescued in the Ednrb -/-;DBH-Ednrb mice. Thus, the results of this study indicate a novel role of Ednrb expressed in SGNs distinct from that in melanocytes in the SV contributing partially to postnatal hearing development.",
author = "Michiru Ida-Eto and Nobutaka Ohgami and Machiko Iida and Ichiro Yajima and Kumasaka, {Mayuko Y.} and Kazutaka Takaiwa and Takashi Kimitsuki and Michihiko Sone and Tsutomu Nakashima and Toyonori Tsuzuki and Shizuo Komune and Masashi Yanagisawa and Masashi Kato",
year = "2011",
month = "8",
day = "26",
doi = "10.1074/jbc.M111.236802",
language = "English (US)",
volume = "286",
pages = "29621--29626",
journal = "Journal of Biological Chemistry",
issn = "0021-9258",
publisher = "American Society for Biochemistry and Molecular Biology Inc.",
number = "34",

}

TY - JOUR

T1 - Partial requirement of endothelin receptor B in spiral ganglion neurons for postnatal development of hearing

AU - Ida-Eto, Michiru

AU - Ohgami, Nobutaka

AU - Iida, Machiko

AU - Yajima, Ichiro

AU - Kumasaka, Mayuko Y.

AU - Takaiwa, Kazutaka

AU - Kimitsuki, Takashi

AU - Sone, Michihiko

AU - Nakashima, Tsutomu

AU - Tsuzuki, Toyonori

AU - Komune, Shizuo

AU - Yanagisawa, Masashi

AU - Kato, Masashi

PY - 2011/8/26

Y1 - 2011/8/26

N2 - Impairments of endothelin receptor B (Ednrb/EDNRB) cause the development of Waardenburg-Shah syndrome with congenital hearing loss, hypopigmentation, and megacolon disease in mice and humans. Hearing loss in Waardenburg- Shah syndrome has been thought to be caused by an Ednrbmediated congenital defect of melanocytes in the stria vascularis (SV) of inner ears. Here we show that Ednrb expressed in spiral ganglion neurons (SGNs) in inner ears is required for postnatal development of hearing in mice. Ednrb protein was expressed in SGNs from WT mice on postnatal day 19 (P19), whereas it was undetectable in SGNs from WT mice on P3. Correspondingly, Ednrb homozygously deleted mice (Ednrb -/- mice) with congenital hearing loss showed degeneration of SGNs on P19 but not on P3. The congenital hearing loss involving neurodegeneration of SGNs as well as megacolon disease in Ednrb -/- mice were markedly improved by introducing an Ednrb transgene under control of the dopamine β-hydroxylase promoter (Ednrb -/-;DBH-Ednrb mice) on P19. Neither defects of melanocytes nor hypopigmentation in the SV and skin in Ednrb -/- mice was rescued in the Ednrb -/-;DBH-Ednrb mice. Thus, the results of this study indicate a novel role of Ednrb expressed in SGNs distinct from that in melanocytes in the SV contributing partially to postnatal hearing development.

AB - Impairments of endothelin receptor B (Ednrb/EDNRB) cause the development of Waardenburg-Shah syndrome with congenital hearing loss, hypopigmentation, and megacolon disease in mice and humans. Hearing loss in Waardenburg- Shah syndrome has been thought to be caused by an Ednrbmediated congenital defect of melanocytes in the stria vascularis (SV) of inner ears. Here we show that Ednrb expressed in spiral ganglion neurons (SGNs) in inner ears is required for postnatal development of hearing in mice. Ednrb protein was expressed in SGNs from WT mice on postnatal day 19 (P19), whereas it was undetectable in SGNs from WT mice on P3. Correspondingly, Ednrb homozygously deleted mice (Ednrb -/- mice) with congenital hearing loss showed degeneration of SGNs on P19 but not on P3. The congenital hearing loss involving neurodegeneration of SGNs as well as megacolon disease in Ednrb -/- mice were markedly improved by introducing an Ednrb transgene under control of the dopamine β-hydroxylase promoter (Ednrb -/-;DBH-Ednrb mice) on P19. Neither defects of melanocytes nor hypopigmentation in the SV and skin in Ednrb -/- mice was rescued in the Ednrb -/-;DBH-Ednrb mice. Thus, the results of this study indicate a novel role of Ednrb expressed in SGNs distinct from that in melanocytes in the SV contributing partially to postnatal hearing development.

UR - http://www.scopus.com/inward/record.url?scp=80051929865&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=80051929865&partnerID=8YFLogxK

U2 - 10.1074/jbc.M111.236802

DO - 10.1074/jbc.M111.236802

M3 - Article

C2 - 21715336

AN - SCOPUS:80051929865

VL - 286

SP - 29621

EP - 29626

JO - Journal of Biological Chemistry

JF - Journal of Biological Chemistry

SN - 0021-9258

IS - 34

ER -