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Planar Cell Polarity Defects and Hearing Loss in Sperm-Associated Antigen 6 (Spag6)-Deficient Mice
American Journal of Physiology-Cell Physiology ( IF 5.5 ) Pub Date : 2020-11-11 , DOI: 10.1152/ajpcell.00166.2020
Xiaofei Li 1 , Daogong Zhang 1 , Lei Xu 1 , Yuechen Han 1 , Wenwen Liu 1 , Wei Li 2 , Zhaomin Fan 1 , Richard M Costanzo 3 , Jerome F Strauss Iii 4 , Zhibing Zhang 2, 5 , Haibo Wang 1
Affiliation  

Spag6 encodes an axoneme central apparatus protein that is required for normal flagellar and cilia motility. Recent findings suggest that Spag6 also plays a role in ciliogenesis, orientation of cilia basal feet, and planar polarity. Sensory cells of the inner ear display unique structural features that underlie their mechanosensitivity. They represent a distinctive form of cellular polarity, known as planar cell polarity (PCP). However, a role for Spag6 in the inner ear has not yet been explored. In the present study, the function of Spag6 in the inner ear was examined using Spag6-deficient mice. Our results demonstrate hearing loss in the Spag6 mutants, associated with abnormalities in cellular patterning, cell shape, stereocilia bundles and basal bodies, as well as abnormally distributed Frizzled class receptor 6 (FZD6), suggesting that Spag6 participates in PCP regulation. Moreover, we found that the sub-apical microtubule meshwork was disrupted. Our observations suggest new functions for Spag6 in hearing and PCP in the inner ear.

中文翻译:

精子相关抗原 6 (Spag6) 缺陷小鼠的平面细胞极性缺陷和听力损失

Spag6 编码正常鞭毛和纤毛运动所需的轴丝中枢装置蛋白。最近的研究结果表明,Spag6 在纤毛发生、纤毛基脚方向和平面极性中也发挥作用。内耳的感觉细胞显示出独特的结构特征,这是其机械敏感性的基础。它们代表了一种独特的细胞极性形式,称为平面细胞极性 (PCP)。然而,尚未探索 Spag6 在内耳中的作用。在本研究中,使用 Spag6 缺陷小鼠检查了 Spag6 在内耳中的功能。我们的结果表明 Spag6 突变体的听力损失与细胞模式、细胞形状、静纤毛束和基体的异常以及异常分布的卷曲类受体 6 (FZD6) 相关,表明 Spag6 参与 PCP 调节。此外,我们发现亚根尖微管网被破坏。我们的观察表明 Spag6 的听力和内耳 PCP 的新功能。
更新日期:2020-11-12
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