Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness.
Science
; 285(5432): 1408-11, 1999 Aug 27.
Article
em En
| MEDLINE
| ID: mdl-10464101
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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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Potássio
/
Fatores de Transcrição
/
Ducto Coclear
/
Surdez
/
Proteínas de Ligação a DNA
/
Proteínas do Tecido Nervoso
Tipo de estudo:
Prognostic_studies
Limite:
Animals
Idioma:
En
Ano de publicação:
1999
Tipo de documento:
Article