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American Journal of Pathology, Vol 98, 385-394, Copyright © 1980 by American Society for Investigative Pathology

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Exocytosis of residual bodies in a lysosomal storage disease

JF Munnell and LC Cork

Scanning and transmission electron-microscopic study of the livers of 2 kittens suffering from GM2 gangliosidosis revealed discontinuities in the plasma membrane of hepatocytes and Kupffer cells, indicating an attempt by the cells to free themselves of excess lysosomal residues by means of exocytosis. Inclusions morphologically similar to those seen in the hepatocytes were observed in the perisinusoidal space. Openings were seen on all surfaces of the hepatocytes. Such extrusion of lysosomal residues is an example of a mechanism rarely observed in viable metazoan cells.


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V. Claus, A. Jahraus, T. Tjelle, T. Berg, H. Kirschke, H. Faulstich, and G. Griffiths
Lysosomal Enzyme Trafficking between Phagosomes, Endosomes, and Lysosomes in J774 Macrophages. ENRICHMENT OF CATHEPSIN H IN EARLY ENDOSOMES
J. Biol. Chem., April 17, 1998; 273(16): 9842 - 9851.
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Copyright © 1980 by the American Society for Investigative Pathology.