Inner ear defects induced by null mutation of the isk gene

Douglas E. Vetter, Jeffrey R. Mann, Philine Wangemann, Jianzhong Liu, K. John McLaughlin, Florian Lesage, Daniel C. Marcus, Michel Lazdunski, Stephen F. Heinemann, Jacques Barhanin

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315 Citations (Scopus)

Abstract

The isk gene is expressed in many tissues. Pharmacological evidence from the inner ear suggests that Isk mediates potassium secretion into the endolymph. To examine the consequences of IsK null mutation on inner ear function, and to produce a system useful for examining the role(s) IsK plays elsewhere, we have produced a mouse strain that carries a disrupted isk locus. Knockout mice exhibit classic shaker/waltzer behavior. Hair cells degenerate, but those of different inner ear organs degenerate at different times. Functionally, we show that in mice lacking isk, the strial marginal cells and the vestibular dark cells of the inner ear are unable to generate an equivalent short circuit current in vitro, indicating a lack of transepithelial potassium secretion.

Original languageEnglish
Pages (from-to)1251-1264
Number of pages14
JournalNeuron
Volume17
Issue number6
DOIs
Publication statusPublished - 1 Jan 1996
Externally publishedYes

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