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Mannosidosis: ocular lesions in the bovine model
R D Jolly1, A Shimada, R R Dalefield
1Department of Veterinary Pathology and Public Health, Massey University, Palmerston North, New Zealand.
Current Eye Research
|September 1, 1987
Summary
Mannosidosis causes widespread cell vacuolation in the bovine eye, affecting the cornea and retina. These vacuoles, likely secondary lysosomes, may explain the ocular opacities observed in human mannosidosis patients.
Area of Science:
- Ocular Pathology
- Lysosomal Storage Diseases
- Animal Models
Background:
- Mannosidosis is a rare genetic lysosomal storage disease.
- Ocular manifestations are significant in human mannosidosis.
- The bovine model offers a valuable system for studying this pathology.
Purpose of the Study:
- To investigate the ocular pathology of mannosidosis using a bovine model.
- To characterize the cellular changes in the bovine eye associated with mannosidosis.
- To correlate observed lesions with human disease manifestations.
Main Methods:
- Histopathological examination of bovine ocular tissues.
- Electron microscopy to identify vacuole characteristics.
- Comparative analysis with known human pathology.
Main Results:
- Widespread vacuolation observed in corneal epithelium, endothelium, fibroblasts, lens epithelium and fibers, and neuroretina.
- Electron microscopy revealed membrane-bound vesicles, consistent with secondary lysosomes.
- Dilatations between lens epithelial cells and fibers were also noted.
Conclusions:
- The bovine model exhibits significant ocular vacuolation in mannosidosis.
- Observed lesions, particularly secondary lysosomes, are implicated in the pathology.
- Further research is needed to definitively link these bovine ocular lesions to human corneal and lens opacities.