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Time course of inner ear degeneration and deafness in mice lacking the Kir4.1 potassium channel subunit

Rozengurt, Nora and Lopez, Ivan and Chiu, Chi-Sung and Kofuji, Paulo and Lester, Henry A. and Neusch, Clemens (2003) Time course of inner ear degeneration and deafness in mice lacking the Kir4.1 potassium channel subunit. Hearing Research, 177 (1-2). pp. 71-80. ISSN 0378-5955. doi:10.1016/s0378-5955(02)00799-2.

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The Kir4.1 gene (KCNJ10) encodes an inwardly rectifying K⁺ channel subunit abundantly expressed in the CNS. Its expression in the mammalian inner ear has been suggested but its function in vivo in the inner ear is unknown. Because diverse human hereditary deafness syndromes are associated with mutations in K⁺ channels, we examined auditory function and inner ear structure in mice with a genetically inactivated Kir4.1 K⁺ channel subunit. Startle response experiments suggest that Kir4.1−/− mice are profoundly deaf, whereas Kir4.1+/− mice react like wild-type mice to acoustic stimuli. In Kir4.1−/− mice, the Reissner membrane is collapsed, the tectorial membrane is swollen, and type I hair cells and spiral ganglion neurons as well as their central processes degenerate over the first postnatal weeks. In the vestibular ganglia, neuronal cell death with apoptotic features is also observed. Immunostaining reveals that Kir4.1 is strongly expressed in stria vascularis of wild-type but not Kir4.1−/− mice. Within the spiral ganglion, Kir4.1 labeling was detected on satellite cells surrounding spiral ganglion neurons and axons. We conclude that Kir4.1 is crucial for normal development of the cochlea and hearing, via two distinct aspects of extracellular K⁺ homeostasis: (1) in stria vascularis, Kir4.1 helps to generate the cochlear endolymph; and (2) in spiral and vestibular ganglia, Kir4.1 in surrounding glial cells helps to support the spiral and vestibular ganglion neurons and their projecting axons.

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Lester, Henry A.0000-0002-5470-5255
Additional Information:© 2003 Elsevier Science B.V. Received 6 September 2001, Accepted 20 December 2002, Available online 12 February 2003.
Subject Keywords:Deafness; Inner ear; Stria vascularis; Inwardly rectifying K+ channel; Development; KCNJ10
Issue or Number:1-2
Record Number:CaltechAUTHORS:20200612-075804212
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Official Citation:Nora Rozengurt, Ivan Lopez, Chi-Sung Chiu, Paulo Kofuji, Henry A Lester, Clemens Neusch, Time course of inner ear degeneration and deafness in mice lacking the Kir4.1 potassium channel subunit, Hearing Research, Volume 177, Issues 1–2, 2003, Pages 71-80, ISSN 0378-5955, (
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:103861
Deposited By: Tony Diaz
Deposited On:12 Jun 2020 17:27
Last Modified:16 Nov 2021 18:26

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