Virchows Archiv B

, 39:187 | Cite as

Lysosomal glycogen storage mimicking the cytological picture of Pompe’s disease as induced in rats by injection of an α-glucosidase inhibitor

II. Alterations in kidney, adrenal gland, spleen and soleus muscle
  • Renate Lüllmann-Rauch
Article
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Summary

This paper describes the tissue distribution of a glycogenosis induced experimentally in rats by intraperitoneal injections of a competitive α-glucosidase inhibitor (Acarbose, Bay g 5421, a pseudotetrasaccharide) which has been used previously to produce glycogenosis in the rat liver (Lüllmann-Rauch 1981a). Among the tissues presently examined, intralysosomal glycogen storage was most prominent (though less severe than in the liver), in the kidney (collecting ducts, renal pelvic transitional epithelium, distal convoluted tubules), in the adrenal cortex (zona reticularis), and in the spleen (trabecular smooth muscle cells). Storage was slight in the adrenal medulla and in the soleus muscle, and hardly apparent in cardiac ventricular muscle. Lysosomal glycogen storage was absent from renal proximal convoluted tubules and from the neurons examined (dorsal root ganglia, supraoptic nucleus).

At the single cell-level, the experimentally induced alterations closely resemble those occurring in inherited glycogenosis type II (Pompe’s disease) in man. Regarding the tissue distribution, there are several differences between the inherited and the induced storage disorder, probably because the latter is determined not only by the glycogen load imposed physiologically on the lysosomal apparatus of a given cell, but also by the pharmacokinetics of the enzyme inhibitor. In spite of these differences, the present experimental model may be useful for investigating cytological problems of glycogen turnover and certain cellular and therapeutic problems of Pompe’s disease.

Key words

Glycogenosis Lysosomes α-Glucosidase inhibitor Kidney Rat 
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Copyright information

© Springer-Verlag 1982

Authors and Affiliations

  • Renate Lüllmann-Rauch
    • 1
  1. 1.Department of AnatomyUniversity of KielKielFederal Republic of Germany

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