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First published online April 11, 2008

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Tbx18 charges cochlea for sound

The cochlea translates mechanical sound into electrical stimulus. Crucial to this is the voltage potential between the sensory hair cells and the cochlea endolymph, in which the correct ionic composition is maintained by fibrocytes in the cochlea wall. Now Andreas Kispert and colleagues report that otic fibrocyte differentiation requires the T-box transcription factor Tbx18 - without it, the endocochlear potential essential for sound conduction by sensory hair cells breaks down and profound deafness occurs (see p. 1725). As Tbx18-null mice die soon after birth owing partly to defective somite development, the researchers studied transgenic msd::Tbx18 mice, which express Tbx18 throughout the presomitic and somitic mesoderm but not the inner ear. These mice are deaf, have abnormal otic mesenchyme compartmentalization and defective otic fibrocyte differentiation, which might, the authors argue, be secondary to Tbx18's earlier role in otic mesenchyme compartmentalization. These findings highlight the crucial role of non-epithelial otic cell types in normal hearing and in the aetiology of deafness.

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Related articles in Development:

Deafness in mice lacking the T-box transcription factor Tbx18 in otic fibrocytes

Mark-Oliver Trowe, Hannes Maier, Michaela Schweizer, and Andreas Kispert
Development 2008 135: 1725-1734. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in Development Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?