中文摘要：

目的：研究口砚晶体蛋白基因敲除对于小鼠晶状体功能的影响。方法：采用胚胎干细胞打靶技术，培育出基因敲除小鼠。用裂隙灯显做镜观察小鼠晶状体的病变；透射和扫描电镜观察品状体的超微结构；观察该基因敲除后晶状体形态和功能改变。结果：βB2晶体蛋白基因敲除后，新生的小鼠晶状体发育正常；随年龄增长，小鼠晶状体的质量、直径和正常小鼠比较明显减少；小鼠在生后数月内发生皮质性白内障，其程度随年龄增长而加重；电镜观察显示晶状体细胞纤维排列异常。结论：小鼠βB2晶体蛋白基因敲除导致年龄相关性白内障的发生。

英文摘要：

AIM： To study the effect on lens by generating mice with a targeted disruption of βB2. METHODS： Gene targeting in embryonic stem cells was used to generate mouse lines in which βB2 was deleted. Knockout mice were screened for lens abnormality with slit lamp biomicroscopy. Microstructure of lens was analyzed by scanning and transmission electron microscopy. The effect on lens morphology and function was observed. RESULTS： The lens appeared to develop normally in the first months of life. In older animals, the weight and axial diameter of the lenses of knockout mice were significantly smaller than those in wild type. Cataracts were formed in the cortex several months after birth and cataract severity increase with age. Electron microscopy showed that the fiber cells were irregularly aligned. CONCLUSION： Cataracts are formed in the cortex several months after birth and cataract severity increase with age.