Torikata C, Kijimoto C, Koto M
Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
Am J Pathol. 1991 Feb;138(2):341-7.
The WIC-Hyd rat is a mutant from the Csk: Wistar-Imamichi rat, with spontaneous hydrocephalus. In male rats, the hydrocephalus is severe and about one half of hydrocephalic male littermates possess situs inversus totalis. Ependymal cilia in these animals are immotile, and this defect is regarded as a mechanical cause of hydrocephalus. This paper presents the ultrastructural features of respiratory cilia in these rats in comparison with those in human immotile cilia syndrome. The respiratory cilia in these rats also are immotile and the dynein arms are missing, as in human cases. Previously only eight dogs with immotile cilia syndrome and a mutant hydrocephalic-polydactyl mouse were reported with respect to these phenomena. However the WIC-Hyd rat is the first useful animal model for human immotile cilia syndrome, and further studies may serve to clarify the genetic background of this condition.
WIC-Hyd大鼠是Csk:Wistar-Imamichi大鼠的突变体，患有自发性脑积水。在雄性大鼠中，脑积水严重，约一半患有脑积水的雄性同窝仔鼠存在完全性内脏反位。这些动物的室管膜纤毛不能运动，这种缺陷被认为是脑积水的一个机械性原因。本文将这些大鼠的呼吸道纤毛超微结构特征与人类不动纤毛综合征患者的进行了比较。这些大鼠的呼吸道纤毛同样不能运动，且如人类病例一样缺少动力蛋白臂。此前，关于这些现象，仅报道过8只患有不动纤毛综合征的犬以及一只患有脑积水-多指畸形的突变小鼠。然而，WIC-Hyd大鼠是首个用于研究人类不动纤毛综合征的有用动物模型，进一步的研究可能有助于阐明这种疾病的遗传背景。
