Hess H H, Newsome D A, Knapka J J, Westney G E
Invest Ophthalmol Vis Sci. 1983 May;24(5):654-7.
Various types of hereditary retinal degeneration have associated posterior subcapsular cataract (PSC). It has been claimed that in the Royal College of Surgeons (RCS) rat model of hereditary retinal dystrophy, the cataract is manifested unpredictably and does not display Mendelian inheritance. It ws shown previously, however, that 100% of pink-eyed retinal dystrophic RCS rats had an onset of bilateral PSC at 7 to 8 weeks of postnatal age, and by 9 to 11 months, 23% of the animals had cataracts visible to the unaided eye. The congenic black-eyed retinal dystrophic RCS rat, however, is a better model for the generally more pigmented human eye. In the present work, it was found that 100% of black-eyed RCS rats had bilateral slit-lamp-detectable PSC beginning at 8 weeks of postnatal age, just as the pink-eyed rats did, despite the fact that dark-eye pigmentation is associated with a 10- to 35-day delay in the rate of degeneration in retinal areas other than the peripheral part of the inferior hemisphere. A higher incidence of mature cataracts in pink-eyed rats (23%) as compared with black-eyed rats (3%) suggests that the amount or intensity of light reaching the lens, retina, and pigmented epithelium may influence maturation of the cataract. However, if light is important in initiating the PSC, its influence was not decreased by dark pigmentation of the eye. RCS rats may be a model for an early onset type of human autosomal recessive retinal degeneration having a constant association of PSC.
多种类型的遗传性视网膜变性都伴有后囊下白内障(PSC)。据称,在遗传性视网膜营养不良的皇家外科学院(RCS)大鼠模型中,白内障的表现不可预测,且不呈现孟德尔遗传。然而,先前的研究表明,100%的红眼视网膜营养不良RCS大鼠在出生后7至8周出现双侧PSC,到9至11个月时,23%的动物出现肉眼可见的白内障。然而,同基因的黑眼视网膜营养不良RCS大鼠对于通常色素沉着更多的人眼来说是一个更好的模型。在本研究中发现,100%的黑眼RCS大鼠在出生后8周开始出现双侧裂隙灯可检测到的PSC,与红眼大鼠情况相同,尽管黑眼色素沉着与下半球周边区域以外的视网膜区域变性速率延迟10至35天有关。与黑眼大鼠(3%)相比,红眼大鼠成熟白内障的发生率更高(23%),这表明到达晶状体、视网膜和色素上皮的光量或强度可能会影响白内障的成熟。然而,如果光在引发PSC方面很重要,那么眼睛的深色色素沉着并不会减弱其影响。RCS大鼠可能是一种人类常染色体隐性视网膜变性早期发病类型的模型,这种类型常伴有PSC。