Establishment and characterization of mouse models for hearing loss based on Molecular interaction

为了建立新的小鼠耳聋模型，明确耳聋的分子机制，我们对耳聋相关蛋白进行了定位克隆、内耳毛细胞表达分析和蛋白-蛋白相互作用研究。2) 4.1B和gelsolin可能是对纤毛成熟起重要作用的myosin XV-whirlin复合体的成员。3) Sans和Whrlin突变体的双纯合子表型数据表明，Sans与Whrlin的功能相互作用对毛细胞立体纤毛的平面细胞极性起调节作用。

To establish new mouse models for deafness and identify molecular mechanism of hearing impairment, we carried out positional cloning approach, expression analysis of the inner ear hair cells and protein-protein interaction studies among the deafness associated-proteins. 1) We identified that responsible gene of two new spontaneous mutants is myosin VI. 2) 4.1B and gelsolin may be members of the myosin XV-whirlin complex that is important for stereocilia maturation. 3) Phenotypic data of double homozygotes between Sans and Whrlin mutants suggest that the function of SANS interacts with WHRLIN and the interaction play regulation of planar cell polarity in stereocilia of the hair cells.