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

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Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV. / Rickheit, Gesa; Maier, Hannes; Strenzke, Nicola; Andreescu, Corina E; Zeeuw, De; Münscher, Adrian; Muenscher, Adrian; Zdebik, Anselm A; Jentsch, Thomas J.

In: EMBO J, Vol. 27, No. 21, 21, 05.11.2008, p. 2907-2917.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Rickheit, G, Maier, H, Strenzke, N, Andreescu, CE, Zeeuw, D, Münscher, A, Muenscher, A, Zdebik, AA & Jentsch, TJ 2008, 'Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV.', EMBO J, vol. 27, no. 21, 21, pp. 2907-2917. https://doi.org/10.1038/emboj.2008.203

APA

Rickheit, G., Maier, H., Strenzke, N., Andreescu, C. E., Zeeuw, D., Münscher, A., Muenscher, A., Zdebik, A. A., & Jentsch, T. J. (2008). Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV. EMBO J, 27(21), 2907-2917. [21]. https://doi.org/10.1038/emboj.2008.203

Vancouver

Rickheit G, Maier H, Strenzke N, Andreescu CE, Zeeuw D, Münscher A et al. Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV. EMBO J. 2008 Nov 5;27(21):2907-2917. 21. https://doi.org/10.1038/emboj.2008.203

Bibtex

@article{38b49a3f79a14f25b5d1d813e90e09d1,
title = "Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV.",
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 = "Animals, Bartter Syndrome, Chloride Channels, Cochlea, DNA-Binding Proteins, Deafness, Endolymph, Evoked Potentials, Gene Deletion, Hair Cells, Auditory, High Mobility Group Proteins, Humans, Integrases, Membrane Proteins, Mice, Mice, Inbred C57BL, Mice, Knockout, Models, Biological, SOXE Transcription Factors, Stria Vascularis, Transcription Factors, Vestibule, Labyrinth",
author = "Gesa Rickheit and Hannes Maier and Nicola Strenzke and Andreescu, {Corina E} and De Zeeuw and Adrian M{\"u}nscher and Adrian Muenscher and Zdebik, {Anselm A} and Jentsch, {Thomas J}",
year = "2008",
month = nov,
day = "5",
doi = "10.1038/emboj.2008.203",
language = "English",
volume = "27",
pages = "2907--2917",
journal = "EMBO J",
issn = "0261-4189",
publisher = "NATURE PUBLISHING GROUP",
number = "21",

}

RIS

TY - JOUR

T1 - Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV.

AU - Rickheit, Gesa

AU - Maier, Hannes

AU - Strenzke, Nicola

AU - Andreescu, Corina E

AU - Zeeuw, De

AU - Münscher, Adrian

AU - Muenscher, Adrian

AU - Zdebik, Anselm A

AU - Jentsch, Thomas J

PY - 2008/11/5

Y1 - 2008/11/5

N2 - 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.

AB - 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.

KW - Animals

KW - Bartter Syndrome

KW - Chloride Channels

KW - Cochlea

KW - DNA-Binding Proteins

KW - Deafness

KW - Endolymph

KW - Evoked Potentials

KW - Gene Deletion

KW - Hair Cells, Auditory

KW - High Mobility Group Proteins

KW - Humans

KW - Integrases

KW - Membrane Proteins

KW - Mice

KW - Mice, Inbred C57BL

KW - Mice, Knockout

KW - Models, Biological

KW - SOXE Transcription Factors

KW - Stria Vascularis

KW - Transcription Factors

KW - Vestibule, Labyrinth

U2 - 10.1038/emboj.2008.203

DO - 10.1038/emboj.2008.203

M3 - SCORING: Journal article

C2 - 18833191

VL - 27

SP - 2907

EP - 2917

JO - EMBO J

JF - EMBO J

SN - 0261-4189

IS - 21

M1 - 21

ER -