AN ULTRASTRUCTURAL-STUDY ON RETINAL NEURAL AND PIGMENT EPITHELIAL-CELLS IN OVINE NEURONAL CEROID-LIPOFUSCINOSIS

被引：7

作者：

GOEBEL, HH

DOPFMER, I

机构：

[1] Division of Neuropathology, University of Mainz, Mainz

[2] Department of Veterinary Pathology and Public Health, Massey University, Palmerston North

来源：

OPHTHALMIC PAEDIATRICS AND GENETICS | 1990年 / 11卷 / 01期

关键词：

Electron microscopy; Lipopigments; Neuronal ceroid-Iipofuscinosis; Photoreceptor; Retinopathy; Sheep;

D O I：

10.3109/13816819009012949

中图分类号：

R77 [眼科学];

学科分类号：

100212 ;

摘要：

Ovine neuronal ceroid-lipofuscinosis represents another well studied model for human neuronal ceroid-Iipofuscinosis (NCL). Accumulation of abnormal lipopigments in various retinal neurons, and loss of photoreceptors are similar to the lesions in human juvenile NCL and indicate that the sheep is a suitable model in which to study the pathogenesis of both NCL lipopig-ment formation and retinopathia pigmentosa. However, this latter process is not as advanced in NCL-diseased sheep as in human patients but far more obvious than in canine NCL in which retinopathy cannot be unequivocally documented. Ovine NCL shares with canine NCL peculiar lamellar inclusions in retinal pigment epithelial cells which may indicate an impaired catabolism of phagocytosed photoreceptor outer segments. As the peculiar lamellar inclusions seen in RPE cells of both NCL-diseased sheep and dogs do not very much resemble the phagocytosed photoreceptor outer segments, it may be assumed that in NCL the final steps in complete removal of these phagocytosed photoreceptor outer segments are also defective. © 1990 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.

引用

页码：61 / 69

页数：9

共 16 条

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