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Clinical,neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis |
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| Authors: | J. Alroy A. Bachrach J. G. Thalhammer N. Panjwani R. Richard R. DeGasperi C. D. Warren D. M. Albert S. S. Raghavan |
| Affiliation: | 1. Department of Pathology, Tufts University School of Medicine and Veterinary Medicine, Boston, USA 2. Department of Medicine, Tufts University School of Veterinary Medicine, Boston, MA 3. Department of Ophthalmology, Tufts University School of Medicine, Boston, MA 4. Laboratory for Carbohydrate Research, Massachusetts General Hospital, Boston, MA 5. Ocular Pathology Laboratory, Massachusetts Eye & Ear Infirmary and Harvard Medical School, Boston, MA 6. Department of Biochemistry, Eunice Shriver Center for Mental Retardation, Waltham, MA |
| Abstract: | The clinical, neurophysiological, morphological and biochemical manifestations of eyes from Persian kittens affected with α-mannosidosis were studied. Clinically the disease is characterized by progressive corneal and lenticular opacification. In addition there is asymmetry in shape and latency of signal conductions which were demonstrated by visual evoked potential studies. Morphological and histochemical studies revealed vacuolization of various ocular cell types which stained positively withConcanavalia ensiformis agglutinin (Con A) and wheat germ agglutinin (WGA). Biochemical studies illustrated low activity of acid α-mannosidase in cultured keratocytes and abnormal storage of partially degraded oligosaccharides in these cells, in vitreous humor and lens. This comprehensive study of ocular α- mannosidosis demonstrates enzyme deficiency which leads to abnormal storage of oligosaccharides in affected cells and is manifested by morphological alterations and functional impairment. |
| Keywords: | Cats eyes α -Mannosidosis |
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