BAIAP2L2 is required for the maintenance of mechanotransducing stereocilia of cochlear hair cells,Journal of Cellular Physiology

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BAIAP2L2 is required for the maintenance of mechanotransducing stereocilia of cochlear hair cells
Journal of Cellular Physiology ( IF 4.5 ) Pub Date : 2021-08-04 , DOI: 10.1002/jcp.30545
Keji Yan ₁ , Wen Zong ₂ , Haibo Du ₁ , Xiaoyan Zhai ₁ , Rui Ren ₁ , Shuang Liu ₃ , Wei Xiong ₃ , Yanfei Wang ₁ , Zhigang Xu _{1,

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Affiliation

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.

中文翻译：

BAIAP2L2 是维持耳蜗毛细胞机械传导静纤毛所必需的

立体纤毛是内耳毛细胞的基于肌动蛋白的细胞突起，在机械电转导 (MET) 中发挥重要作用。静纤毛被组织成几排高度增加的 MET 蛋白复合物，位于较短排静纤毛的尖端。在较短的行机械转导静纤毛的尖端还存在所谓的“第 2 行蛋白质复合物”，其功能障碍导致机械转导静纤毛的退化。在目前的工作中，我们表明 BAIAP2L2 定位于新生和成年小鼠耳蜗毛细胞中较短行静纤毛的尖端。Baiap2l2失活导致机械传导静纤毛退化，最终导致任何性别小鼠的严重听力损失。始终如一地，电生理学和 FM 1-43FX 染料摄取结果证实 MET 电流在Baiap2l2敲除小鼠中受到损害。此外，BAIAP2L2 与已知的第 2 行复合成分 EPS8L2、TWF2 和 CAPZB2 结合，CAPZB2 的静纤毛尖端定位依赖于功能性 BAIAP2L2。有趣的是，BAIAP2L2 还与已知的 MET 复合成分 CIB2 结合，BAIAP2L2 的纤毛尖端定位在 Cib2 中被取消敲除老鼠。总之，我们目前的数据表明，BAIAP2L2 是第 2 行复杂成分，是维持机械转导静纤毛所必需的。同时，特定的 MET 成分如 CIB2 可能通过与 BAIAP2L2 结合在静纤毛维持中发挥直接作用。

更新日期：2021-08-04

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