The DOI matches its Crossref record.
Altered Cochlear Fibrocytes in a Mouse Model of DFN3 Nonsyndromic Deafness
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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Sugitani, Y., Nakai, S., Minowa, O., Nishi, M., Jishage, K.-I., Kawano, H., Mori, K., Ogawa, M., & Noda, T. (2002) · Genes & Development · doi:10.1101/gad.978002 · Cited by 259
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Metadata
- Authors
- Minowa, O., Ikeda, K., Sugitani, Y., Oshima, T., Nakai, S., Katori, Y., Suzuki, M., Furukawa, M., Kawase, T., Zheng, Y., Ogura, M., Asada, Y., Watanabe, K., Yamanaka, H., Gotoh, S., Nishi-Takeshima, M., Sugimoto, T., Kikuchi, T., Takasaka, T., & Noda, T.
- Venue
- Science
- Published
- 1999-08-27
- Volume
- 285
- Issue
- 5432
- Pages
- 1408-1411
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2097960859
- Cited by
- 222
- References
- 0
- FWCI
- 5.98
- Type
- journal article
- Language
- en
- Open access
- closed
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- Minowa, O., Ikeda, K., Sugitani, Y., Oshima, T., Nakai, S., Katori, Y., Suzuki, M., Furukawa, M., Kawase, T., Zheng, Y., Ogura, M., Asada, Y., Watanabe, K., Yamanaka, H., Gotoh, S., Nishi-Takeshima, M., Sugimoto, T., Kikuchi, T., Takasaka, T., & Noda, T. (1999). Altered Cochlear Fibrocytes in a Mouse Model of DFN3 Nonsyndromic Deafness. Science. https://doi.org/10.1126/science.285.5432.1408
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
