Goebel H H, Ikeda K, Eichholtz W, Koppang N, Bergsjø T
Ophthalmic Paediatr Genet. 1985 Feb;5(1-2):3-11. doi: 10.3109/13816818509007849.
An electron microscopic study was performed on eyes of Labrador dogs afflicted with progressive retinal atrophy (PRA). There was complete loss of photoreceptors, atrophy of the remaining retina and gliosis in the peripheral part while the central retina showed incomplete loss of photoreceptors and an almost total disappearance of photoreceptor outer segments. Melanin-bearing cells, largely containing melanolysosomes, were found deep inside the retina. This electron microscopic study also incorporated the retina of a middle-aged woman affected by retinopathia pigmentosa (RP). The fine structure of the diseased retina showed a similar pattern of lesions, more pronounced in the periphery of the retina. Similar electron microscopic findings between the two disease processes render PRA of the Labrador dog a useful model for a comparative study of the development and intraretinal spread of human RP.
对患有进行性视网膜萎缩(PRA)的拉布拉多犬的眼睛进行了电子显微镜研究。光感受器完全丧失,剩余视网膜萎缩,周边部分出现胶质增生,而中央视网膜光感受器不完全丧失,光感受器外段几乎完全消失。在视网膜深处发现了主要含有黑素溶酶体的含黑素细胞。这项电子显微镜研究还纳入了一名患有色素性视网膜炎(RP)的中年女性的视网膜。患病视网膜的精细结构显示出类似的病变模式,在视网膜周边更为明显。这两种疾病过程之间相似的电子显微镜发现使拉布拉多犬的PRA成为人类RP发育和视网膜内扩散比较研究的有用模型。