Sobin A, Anniko M, Flock A
Arch Otorhinolaryngol. 1982;236(1):1-6. doi: 10.1007/BF00464051.
The shaker-2 mouse with inherited inner ear disease suffers from deafness and a shaking-waltzing behavior. The hair cell type I in cristae ampullares and maculae utriculi show a specific pathology, featuring fusion of the stereocilia and presence of a rod-shaped inclusion body. The inclusion body is composed of filaments that could be identified as the protein actin by the method of decoration with subfragment S-1 of myosin. The functional polarity was determined, and S-1 fragments were found to point apically, that is, from the nucleus up toward the cuticular plate. These observations are identical to those earlier described in the waltzing guinea pig. It is concluded that the identical pathology at a cellular level in two different species may indicate a pathologic disorder in a process fundamental to the normal development of this type of hair cell.
患有遗传性内耳疾病的shaker-2小鼠会出现耳聋和摇晃-华尔兹行为。壶腹嵴和椭圆囊斑中的I型毛细胞表现出特定的病理学特征，包括静纤毛融合和存在杆状包涵体。该包涵体由细丝组成，通过用肌球蛋白亚片段S-1进行装饰的方法可将其鉴定为肌动蛋白。确定了功能极性，发现S-1片段指向顶端，即从细胞核向上指向角质板。这些观察结果与早期在华尔兹豚鼠中描述的结果相同。结论是，两个不同物种在细胞水平上的相同病理学可能表明这种类型毛细胞正常发育的一个基本过程中存在病理紊乱。
