携带嵌合型或突变型III型中间丝（IF）基因的转基因小鼠。
Transgenic mice carrying chimeric or mutated type III intermediate filament (IF) genes.
作者信息
Bloemendal H, Raats J M, Pieper F R, Benedetti E L, Dunia I
机构信息
Department of Biochemistry, University of Nijmegen, The Netherlands.
出版信息
Cell Mol Life Sci. 1997 Jan;53(1):1-12. doi: 10.1007/pl00000571.
Mice carrying chimeric, truncated or mutated genes encoding intermediate filament (IF) proteins type III do not show any detectable severe pathology. However, upon (over)expression of the transgene in the eye lens all animals develop lens opacification (cataract). At the cellular level the loss of visual acuity is preceded by interference with the terminal differentiation of lens fibre cells, plasma membrane damage, distorted assembly of the IF cytoskeleton and perturbation of the cytoskeleton-membrane complex. The degree of expression is paralleled by the extent of the damages.
携带编码III型中间丝（IF）蛋白的嵌合、截短或突变基因的小鼠未表现出任何可检测到的严重病理变化。然而，当转基因在眼晶状体中（过度）表达时，所有动物都会出现晶状体混浊（白内障）。在细胞水平上，视力丧失之前会出现晶状体纤维细胞终末分化受到干扰、质膜损伤、IF细胞骨架组装扭曲以及细胞骨架-膜复合体紊乱。表达程度与损伤程度平行。
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