Altered Cochlear Fibrocytes in a Mouse Model of DFN3 Nonsyndromic Deafness
Osamu Minowa(Japan Science and Technology Agency), Katsuhisa Ikeda(Tohoku University), Yoshinobu Sugitani(Japan Science and Technology Agency), Takeshi Oshima(Tohoku University), Shigeyasu Nakai(Japan Science and Technology Agency), Yukio Katori(Tohoku University), Masaaki Suzuki(Tohoku University), Masae Furukawa(Tohoku University), Tetsuaki Kawase(Tohoku University), Yulian Zheng(Tohoku University), Masaki Ogura(Tohoku University), Y. Asada(Tohoku University), Kenichi Watanabe(Tohoku University), Hisashi Yamanaka(Japan Science and Technology Agency), Shimpei Gotoh(Tohoku University), M. Nishi-Takeshima(The University of Tokyo), Tetsuo Sugimoto(Kansai Medical University), Toshihiko Kikuchi(Tohoku University), Tomonori Takasaka(Tohoku University), T Noda(Tohoku University)
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Abstract
DFN3, an X chromosome-linked nonsyndromic mixed deafness, is caused by mutations in the BRN-4 gene, which encodes a POU transcription factor. Brn-4-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.
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