Cohen A I
Invest Ophthalmol Vis Sci. 1983 Jul;24(7):832-43.
As a control for biochemical studies in progress, an ultrastructural study has been carried out on the deteriorating, 21-day photoreceptors of the 020/Cpb strain of mice, homozygous for retinal degeneration, slow (rds). At 21 postnatal days, outer segments were essentially lacking, but cilia erupting from the inner segments were common. A low percentage of cilia bore small cytoplasmic masses containing a few layered membranes, and rare inner segments possessed spherical aggregations of multilayered membranes. Pigment epithelial cells also possessed membranous aggregations in presumed phagosomes. While other parts of photoreceptors possessed the usual organelles of normal rods, inner segments were reduced in volume, and the layer of photoreceptor synaptic terminals was thinner. Mutant 21-day retinas possessed about two-thirds of the protein of normal 21-day retinas but 50% more protein than "rodless" (rd/rd) 21-day retinas. Surprisingly, while dark-adapted rds retinas possessed markedly lower levels of cyclic GMP as compared to controls, light-adaptation significantly reduced cyclic GMP and cyclic AMP levels, and biochemical data point to persistent light-modulated cyclic nucleotide levels in the photoreceptors.
作为正在进行的生化研究的对照,对视网膜退化缓慢(rds)的020/Cpb品系小鼠21日龄退化的光感受器进行了超微结构研究。出生后21天,外段基本缺失,但从内段长出的纤毛很常见。一小部分纤毛带有含有几层膜的小细胞质团块,很少有内段具有多层膜的球形聚集体。色素上皮细胞在假定的吞噬体中也具有膜状聚集体。虽然光感受器的其他部分具有正常视杆细胞通常的细胞器,但内段体积减小,光感受器突触终末层变薄。突变型21日龄视网膜的蛋白质含量约为正常21日龄视网膜的三分之二,但比“无杆”(rd/rd)21日龄视网膜多50%。令人惊讶的是,与对照组相比,暗适应的rds视网膜的环鸟苷酸水平明显较低,而光适应显著降低了环鸟苷酸和环腺苷酸水平,生化数据表明光感受器中存在持续的光调制环核苷酸水平。