Wiechmann A F, Font R L, Hollyfield J G
Department of Neurobiology, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC 27103.
Exp Eye Res. 1991 Mar;52(3):311-20. doi: 10.1016/0014-4835(91)90095-v.
Photoreceptor cell pathology was investigated in an 8-yr-old mixed-breed dog which had displayed visual symptoms of 1 month duration. An electroretinogram detected no light-evoked responses. Light and electron microscopic features showed marked thinning and atrophy of the outer both the tapetal and non-tapetal retina appeared to be involved. In the non-tapetal region, a majority of the rod inner segments were missing, while scattered mitochondria-filled stubby inner segments of cones were readily identified. Inner segments of both rods and cones were observed in the tapetal region. Photoreceptor outer segments were completely absent from the affected retina, and no outer segment debris was observed between the photoreceptor layer and the retinal pigmented epithelium (RPE). Autoradiographic analysis of 3-mm retinal disks from the degenerate retina following incubation with [3H]uridine indicated that only 61% +/- 13 S.D. of the remaining nuclei of rod photoreceptors were undergoing RNA synthesis, whereas more than 99% of cone nuclei incorporated the label. Normal and degenerate retina were also analysed for localization of hydroxyindole-O-methyltransferase (HIOMT)-like immunoreactivity. While the normal retina showed immunoreactivity in both rod and cone photoreceptors with more intense immunoreactivity present in cones, the degenerate retina showed HIOMT-like immunoreactivity only in the remaining cone photoreceptors. The results of this study of idiopathic photoreceptor degeneration of the canine retina suggest that although both photoreceptor types are involved, rods are more severely affected than cones.
对一只8岁的混种犬进行了光感受器细胞病理学研究,该犬出现视觉症状已有1个月。视网膜电图未检测到光诱发反应。光镜和电镜特征显示,视网膜外层明显变薄和萎缩,包括反光层和非反光层视网膜似乎均受累。在非反光层区域,大多数视杆细胞内节缺失,而散在的充满线粒体的短粗视锥细胞内节很容易识别。在反光层区域观察到视杆和视锥细胞的内节。受影响的视网膜中完全没有光感受器外节,在光感受器层和视网膜色素上皮(RPE)之间未观察到外节碎片。用[3H]尿苷孵育退化视网膜后,对3毫米视网膜切片进行放射自显影分析表明,仅6(1%\pm13)标准差的剩余视杆光感受器细胞核正在进行RNA合成,而超过99%的视锥细胞核掺入了标记物。还对正常和退化视网膜进行了羟吲哚-O-甲基转移酶(HIOMT)样免疫反应性的定位分析。正常视网膜在视杆和视锥光感受器中均显示免疫反应性,视锥细胞中的免疫反应性更强,而退化视网膜仅在剩余的视锥光感受器中显示HIOMT样免疫反应性。这项对犬视网膜特发性光感受器退化的研究结果表明,虽然两种光感受器类型均受累,但视杆细胞比视锥细胞受影响更严重。