| First Author | Minowa O | Year | 1999 |
| Journal | Science | Volume | 285 |
| Issue | 5432 | Pages | 1408-11 |
| PubMed ID | 10464101 | Mgi Jnum | J:57298 |
| Mgi Id | MGI:1344188 | Doi | 10.1126/science.285.5432.1408 |
| Citation | Minowa O, et al. (1999) Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness [see comments]. Science 285(5432):1408-11 |
| abstractText | 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. |
