KCNK5 channels mostly expressed in cochlear outer sulcus cells are indispensable for hearing

被引:20
作者
Cazals, Yves [1 ,2 ]
Bevengut, Michelle [3 ,4 ]
Zanella, Sebastien [3 ,4 ,5 ,6 ]
Brocard, Frederic [5 ,6 ]
Barhanin, Jacques [7 ,8 ,9 ]
Gestreau, Christian [3 ,4 ]
机构
[1] Aix Marseille Univ, UMR7260, Federat Rech 3C, Lab Neurosci Integrat & Adaptat, F-13331 Marseille, France
[2] CNRS, F-13331 Marseille, France
[3] Aix Marseille Univ, UMR7286, Ctr Rech Neurobiol & Neurophysiol Marseille, F-13344 Marseille, France
[4] CNRS, F-13344 Marseille, France
[5] Aix Marseille Univ, UMR7289, Inst Neurosci Timone, F-13005 Marseille, France
[6] CNRS, F-13005 Marseille, France
[7] Univ Nice Sophia Antipolis, UMR7370, Lab Physiomed Mol, F-06107 Nice, France
[8] CNRS, F-06107 Nice, France
[9] Labs Excellence Ion Channel Sci & Therapeut, Montpellier, France
关键词
RENAL TUBULAR-ACIDOSIS; DOMAIN POTASSIUM CHANNELS; SPIRAL GANGLION NEURONS; PENDRED-SYNDROME GENE; SYNDROME MOUSE MODEL; INNER-EAR; K+ CHANNEL; CAUSES DEAFNESS; HAIR-CELLS; DOMINANT DEAFNESS;
D O I
10.1038/ncomms9780
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
In the cochlea, K+ is essential for mechano-electrical transduction. Here, we explore cochlear structure and function in mice lacking K+ channels of the two-pore domain family. A profound deafness associated with a decrease in endocochlear potential is found in adult Kcnk5(-/-) mice. Hearing occurs around postnatal day 19 (P19), and completely disappears 2 days later. At P19, Kcnk5(-/-) mice have a normal endolymphatic [K+] but a partly lowered endocochlear potential. Using Lac-Z as a gene reporter, KCNK5 is mainly found in outer sulcus Claudius', Boettcher's and root cells. Low levels of expression are also seen in the spiral ganglion, Reissner's membrane and stria vascularis. Essential channels (KCNJ10 and KCNQ1) contributing to K+ secretion in stria vascularis have normal expression in Kcnk5(-/-) mice. Thus, KCNK5 channels are indispensable for the maintenance of hearing. Among several plausible mechanisms, we emphasize their role in K+ recycling along the outer sulcus lateral route.
引用
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页数:9
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