Affiliation:
1. Faculty of Veterinary Science, Massey University, Palmerston North, New Zealand
Abstract
Mannosidosis of Angus calves is an inherited lethal disease associated with a deficiency of the lysosomal enzyme α-mannosidase. It is characterised by vacuolation of neurons, macrophages, fixed reticuloendothelial cells, exocrine epithelial cells and to a lesser extent a wide variety of other cells. Vacuoles are shown to be membrane-bound vesicles and are considered to be secondary lysosomes in which are stored mannose-containing oligosaccharides that result from incomplete degradation of glycoproteins. Such glycoproteins may enter the lysosomal system by heterophagy, autophagy or crinophagy. The presence in the pancreas of zymogen granules in storage vesicles indicates that incomplete degradation of secretory granules might help explain the widespread vacuolation of other exocrine cells. It is suggested that such granules may enter the lysosomal system by crinophagy.
Reference25 articles.
1. MANNOSIDOSIS: CLINICAL, FINE-STRUCTURAL AND BIOCHEMICAL FINDINGS IN THREE CASES
2. Daems W.T., Van Gemund J.J., Vio P.M.A., Willighagen R.G. J., Den Tandt W.T.: The use of suction biopsy material for the study of lysosomal storage diseases in Lysosomes and Storage Diseases, ed. Hers, Van Hoof; pp. 575–598, Academic Press, New York and London, 1973.
Cited by
66 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献