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首页> 外文期刊>Journal of cellular physiology. >BAIAP2L2 is required for the maintenance of mechanotransducing stereocilia of cochlear hair cells
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BAIAP2L2 is required for the maintenance of mechanotransducing stereocilia of cochlear hair cells

机译：BAIAP2L2需要维护mechanotransducing静纤毛的耳蜗的头发细胞

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摘要

Stereocilia are actin-based cell protrusions of inner ear hair cells that play an essential role in mechano-electrical transduction (MET). Stereocilia are organized into several rows of increasing heights with the MET protein complex localized at the tips of shorter row stereocilia. At the tips of shorter row mechanotransducing stereocilia also resides a so-called "row 2 protein complex" whose dysfunction causes degeneration of the mechanotransducing stereocilia. In the present work, we show that BAIAP2L2 is localized at the tips of shorter row stereocilia in neonatal and adult mouse cochlear hair cells. Baiap2l2 inactivation causes degeneration of the mechanotransducing stereocilia, which eventually leads to profound hearing loss in mice of either sex. Consistently, electrophysiology and FM 1-43FX dye uptake results confirm that MET currents are compromised in Baiap2l2 knockout mice. Moreover, BAIAP2L2 binds to known row 2 complex components EPS8L2, TWF2, and CAPZB2, and the stereociliary tip localization of CAPZB2 is dependent on functional BAIAP2L2. Interestingly, BAIAP2L2 also binds to CIB2, a known MET complex component, and the stereociliary tip localization of BAIAP2L2 is abolished in Cib2 knockout mice. In conclusion, our present data suggest that BAIAP2L2 is a row 2 complex component, and is required for the maintenance of mechanotransducing stereocilia. Meanwhile, specific MET components such as CIB2 might play a direct role in stereocilia maintenance through binding to BAIAP2L2.
机译：静纤毛actin-based细胞突起内耳毛细胞,起着关键作用在mechano-electrical转导(满足)。静纤毛被组织成几行增加高度满足蛋白质复合体本地化的提示短行静纤毛。在短行mechanotransducing的技巧静纤毛也驻留一个所谓的“第二行蛋白质复合体的功能障碍的原因变性的mechanotransducing静纤毛。BAIAP2L2局部短的提示行静纤毛在新生儿和成年小鼠耳蜗毛细胞。变性的mechanotransducing静纤毛,最终导致深远的听力损失在老鼠身上的性。电生理学和调频1-43FX染料吸收结果证实了电流妥协在Baiap2l2基因敲除小鼠。结合已知的第二行EPS8L2复杂的组件,TWF2, CAPZB2, stereociliary小费本地化CAPZB2依赖于功能BAIAP2L2。CIB2,已知遇到复杂的组件,stereociliary提示BAIAP2L2的本地化废除Cib2基因敲除小鼠。我们目前的数据表明,BAIAP2L2第2行复杂的组件,是所需的维护mechanotransducing静纤毛。与此同时,特定的组件(如CIB2会面可能在静纤毛发挥直接作用通过绑定BAIAP2L2维护。

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