Lysosomal glycogen storage mimicking the cytological picture of Pompe's disease as induced in rats by injection of an alpha-glucosidase inhibitor. I. Alterations in liver.
The present paper describes an animal model of lysosomal glycogenosis as induced by a competitive inhibitor of alpha-glucosidase. Rats received intraperitoneal injections of the inhibitor, a pseudotetrasaccharide (Acarbose, Bay g 5421); liver tissue was examined by light and electron microscopy. Substrate-histochemical and enzyme-cytochemical methods were used to demonstrate intralysosomal glycogen storage within hepatocytes and Kupffer cells. The cytological picture closely resembled that occurring in glycogenosis type II (Pompe's disease) of humans. After cessation of drug treatment, the glycogen storage was slowly reversible. The present results point to the physiological role of the lysosomal apparatus for intracellular glycogen turnover. On the cellular level, this experimentally induced glycogenosis may be useful as a model of Pompe's disease.
D020909:Acarbose; D000818:Animals; D005260:Female; D005959:Glucosidases / Q000037:antagonists & inhibitors*; D006008:Glycogen Storage Disease / Q000139:chemically induced*; D006009:Glycogen Storage Disease Type II / Q000139:chemically induced* / Q000473:pathology; D065089:Glycoside Hydrolase Inhibitors; D007728:Kupffer Cells / Q000648:ultrastructure; D008099:Liver / Q000648:ultrastructure*; D008247:Lysosomes / Q000648:ultrastructure; D008854:Microscopy, Electron; D009844:Oligosaccharides; D051381:Rats; D014312:Trisaccharides
D065089:Glycoside Hydrolase Inhibitors; D009844:Oligosaccharides; D014312:Trisaccharides; D005959:Glucosidases; D020909:Acarbose