视紫红质Pro347Leu转基因猪视杆和视锥细胞群退化的病理学研究。
A pathologic study of degeneration of the rod and cone populations of the rhodopsin Pro347Leu transgenic pigs.
作者信息
Tso M O, Li W W, Zhang C, Lam T T, Hao Y, Petters R M, Wong F
机构信息
Chinese University of Hong Kong.
出版信息
Trans Am Ophthalmol Soc. 1997;95:467-79; discussion 479-83.
PURPOSE
Transgenic pigs with rhodopsin (Pro347Leu) mutation exhibited rod-cone degeneration. We compared the pathologic characteristics of the rod degeneration versus those of the cone cells.
METHODS
The posterior and peripheral retinas of these transgenic pigs of age 4, 6, 8, 12, 24 and 33 weeks and normal pigs of age 4 and 8 weeks were studied by light and EM and morphometry.
RESULTS
The pathologic changes observed in the posterior and peripheral retinas of the transgenic pigs could be conveniently described in 3 phases: I) an initial phase of rapid and extensive degeneration of the rod cells in the first 6 weeks of age; II) an acute phase of cone cell degeneration involving approximately half of the population and lingering rod degeneration in the 6 to 12 weeks of age; and III) a partial cone recovery to be followed by a chronic degenerative phase of the remaining cones cells from 12 to 33 weeks of age.
CONCLUSION
Our study showed that the degenerative changes of rod cells could be differentiated from those of the cone cells. Cone and rod populations degenerated along different time schedule with different pathologic features. Hence, treatment for retinitis pigmentosa might vary with the different stages of the disease.
目的
携带视紫红质（Pro347Leu）突变的转基因猪表现出视杆 - 视锥细胞变性。我们比较了视杆细胞变性与视锥细胞变性的病理特征。
方法
对4、6、8、12、24和33周龄的这些转基因猪以及4和8周龄的正常猪的后部和周边视网膜进行了光镜、电镜检查及形态计量学分析。
结果
在转基因猪的后部和周边视网膜中观察到的病理变化可方便地分为三个阶段：I）在出生后前6周视杆细胞迅速广泛变性的初始阶段；II）在6至12周龄时视锥细胞变性的急性期，约一半的视锥细胞受累，同时视杆细胞持续变性；III）在12至33周龄时，部分视锥细胞恢复，随后其余视锥细胞进入慢性变性阶段。
结论
我们的研究表明，视杆细胞的变性变化可与视锥细胞的变性变化相区分。视锥细胞和视杆细胞群体沿不同的时间进程退化，具有不同的病理特征。因此，视网膜色素变性的治疗可能因疾病的不同阶段而异。
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