Article Dans Une Revue Nature Année : 1998

Connexin 26 gene linked to a dominant deafness

Résumé

During the course of a study aimed at identifying inner ear-specific transcripts, a 1,906-bp murine cDNA predicted to encode a secreted 469-aa protein with two domains of homology with the secreted phospholipases A 2 was isolated. This transcript is specifically expressed in the inner ear from embryonic day 9.5. The encoded 95-kDa glycoprotein is the major protein of the utricular and saccular otoconia and thus was named otoconin-95. By immunohistofluorescence, otoconin-95 also was detected in the cupulae of the semicircular canals and in previously undescribed transient granular structures of the cochlea. Otoconin-95 was found to be synthesized by various nonsensory cell types, but not by the supporting cells of the sensory epithelia, which produce the otoconial precursor vesicles. In addition, multiple isoforms generated by differential splicing were observed in different combinations during development. Based on the present results, we propose a model for the formation of the otoconia.

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Dates et versions

pasteur-05019638 , version 1 (03-04-2025)

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Françoise Denoyelle, Genevieve Lina-Granade, Henri Plauchu, Roberto Bruzzone, Hassan Chaïb, et al.. Connexin 26 gene linked to a dominant deafness. Nature, 1998, 393 (6683), pp.319-320. ⟨10.1038/30639⟩. ⟨pasteur-05019638⟩
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