Establishment of rhodopsin transgenic rabbit line and analysis of retinal degeneration

我们利用细菌人工染色体(BAC)转基因技术建立了视网膜色素变性(RP)模型--视紫红质P347L转基因(TG)兔。这些兔表现为杆状显性进行性视网膜变性，光感受器的丢失有明显的区域差异。TG兔视网膜电信号各成分均呈递减趋势，a波的影响大于b波，振荡电位(OPS)保存最好。有趣的是，年轻TG兔的OP波幅显著大于野生型兔。药理学实验表明，幼年TG兔的OPS明显较大是由于视网膜内功能的继发性改变。

We have generated a rabbit model of retinitis pigmentosa (RP), the rhodopsin P347L transgenic (Tg) rabbit, by using bacterial artificial chromosome (BAC) transgenesis. These rabbits showed a rod-dominant, progressive retinal degeneration with a marked regional variations in the loss of photoreceptors. All of the electroretinogram components of the Tg rabbits decrease progressively with the a-wave more affected than the b-wave, and the oscillatory potentials (OPs) were the best preserved. Interestingly, the OP amplitudes of young Tg rabbits were significantly larger than those of wild-type rabbits. Pharmacological experiments showed that the significantly larger OPs in young Tg rabbits resulted from secondary alterations in the inner retinal function.