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Endocochlear potential depends on Cl(-) channels: mechanism underlying deafness in Bartter syndrome IV

Item Type:Article
Title:Endocochlear potential depends on Cl(-) channels: mechanism underlying deafness in Bartter syndrome IV
Creators Name:Rickheit, G. and Maier, H. and Strenzke, N. and Andreescu, C.E. and De Zeeuw, C.I. and Muenscher, A. and Zdebik, A.A. and Jentsch, T.J.
Abstract:Human Bartter syndrome IV is an autosomal recessive disorder characterized by congenital deafness and severe renal salt and fluid loss. It is caused by mutations in BSND, which encodes barttin, a beta-subunit of ClC-Ka and ClC-Kb chloride channels. Inner-ear-specific disruption of Bsnd in mice now reveals that the positive potential, but not the high potassium concentration, of the scala media depends on the presence of these channels in the epithelium of the stria vascularis. The reduced driving force for K(+)-entry through mechanosensitive channels into sensory hair cells entails a profound congenital hearing loss and subtle vestibular symptoms. Although retaining all cell types and intact tight junctions, the thickness of the stria is reduced early on. Cochlear outer hair cells degenerate over several months. A collapse of endolymphatic space was seen when mice had additionally renal salt and fluid loss due to partial barttin deletion in the kidney. Bsnd(-/-) mice thus demonstrate a novel function of Cl(-) channels in generating the endocochlear potential and reveal the mechanism leading to deafness in human Bartter syndrome IV.
Keywords:Anion Transport, Inner Ear, Otoacoustic Emission, Potassium Recycling, Sox10, Animals, Mice
Source:EMBO Journal
ISSN:0261-4189
Publisher:Nature Publishing Group (U.S.A.)
Volume:27
Number:21
Page Range:2907-2917
Date:5 November 2008
Official Publication:https://doi.org/10.1038/emboj.2008.203
PubMed:View item in PubMed

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