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Canine GSD II closely parallels the infantile form of the human disease, except for the presence of oesophageal dilatation. Generalized glycogen storage particularly affected muscular tissues (skeletal, oesophageal, cardiac and smooth muscle). The altered cells showed glycogen accumulation in the cytosol and in autophagic membrane\u2010bound vacuoles (glycogenosomes). They also showed increased acid phosphatase activity consistent with the lysosomal nature of this storage disorder. The cytopathology in canine and human GSD II appears to evolve from segregation of glycogen during regular cellular autophagy, phagolysosomal accumulation of the undigested glycogen, and eventually rupture of distended glycogenosomes. This study indicates that the usefulness of canine GSD II as an animal model of human disease, extends to the area of pathogenesis.<\/jats:p>","DOI":"10.1007\/bf01805484","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T22:39:58Z","timestamp":1119911998000},"page":"38-46","source":"Crossref","is-referenced-by-count":23,"title":["Comparative pathology of the canine model of glycogen storage disease type II (Pompe's disease)"],"prefix":"10.1002","volume":"8","author":[{"given":"H. C.","family":"Walvoort","sequence":"first","affiliation":[{"name":"Laboratory for Pathology National Institute of Public Health and Environmental Hygiene  P.O. Box 1 Bilthoven 3720 BA The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. A. M. A.","family":"Dormans","sequence":"additional","affiliation":[{"name":"Laboratory for Pathology National Institute of Public Health and Environmental Hygiene  P.O. Box 1 Bilthoven 3720 BA The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. S. G. A. M.","family":"van den Ingh","sequence":"additional","affiliation":[{"name":"Department of Veterinary Pathology State University of Utrecht  P.O. Box 80.158 Utrecht 3508 TD The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1984,2,29]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/10.6.741"},{"key":"e_1_2_1_3_1","first-page":"1139","article-title":"An electron microscopic and biochemical study of type II glycogenosis","volume":"13","author":"Baudhuin P.","year":"1964","journal-title":"Lab. 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