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Hepatic ultrastructure in mucolipidosis I (lipomucopolysaccharidosis)

Virchows Archiv B Cell Pathology Zell-pathologie, ISSN: 0042-6431, Vol: 7, Issue: 1, Page: 189-204
1971
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Liver biopsy specimens from two children with mucolipidosis I (lipomucopolysaccharidosis) were studied with the electron microscope. The hepatocytes show membrane-delineated storage vacuoles with evenly distributed reticulogranular structures, opaque globules, tubular structures, lamellar figures and/or vesicles. Lysosomes with islets of storage material may also be seen. Kupffer cells are greatly increased in size and exhibit tightly packed storage vacuoles. Sinusoids are filled with cellular debris, a variety of storage elements and enlarged Kupffer cells. Fibroblast-like cells of portal triads and perisinusoidal spaces may also reveal storage vacuoles. The fine structure of the storage vacuoles found in the hepatocytes supports the hypothesis that storage vacuoles may take their origin from lysosomes. © 1971 Springer-Verlag.

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