Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness

O. Minowa, K. Ikeda, Y. Sugitani, T. Oshima, S. Nakai, Y. Katori, M. Suzuki, M. Furukawa, T. Kawase, Y. Zheng, M. Ogura, Y. Asada, K. Watanabe, H. Yamanaka, S. Gotoh, M. Nishi-Takeshima, T. Sugimoto, T. Kikuchi, T. Takasaka, T. Noda

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175 Citations (Scopus)

Abstract

DFN3, an X chromosome-linked nonsyndromic mixed deafness, is caused by mutations in the BRN-4 gene, which encodes a POU transcription factor. Brn4- deficient mice were created and found to exhibit profound deafness. No gross morphological changes were observed in the conductive ossicles or cochlea, although there was a dramatic reduction in endocochlear potential Electron microscopy revealed severe ultrastructural alterations in cochlear spiral ligament fibrocytes. The findings suggest that these fibrocytes, which are mesenchymal in origin and for which a role in potassium ion homeostasis has been postulated, may play a critical role in auditory function.

Original languageEnglish
Pages (from-to)1408-1411
Number of pages4
JournalScience
Volume285
Issue number5432
DOIs
Publication statusPublished - 1999 Aug 27

ASJC Scopus subject areas

  • General

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