Lysosomal changes and enterocytic copper deposits in Wilson's disease.
In 4 siblings, of whom 2 had symptoms of Wilson's disease, electron microscopy showed that the number and size of lysosomes in the enterocytes were increased compared with controls. Electron-dense aggregates, observed near the lysosomes in the 2 symptomatic patients and 1 other sibling, were shown, by means of a microanalytical method, to contain copper. These findings could suggest a possible role of the small intestine in Wilson's disease.
D003300:Copper / Q000378:metabolism*; D004848:Epithelium / Q000378:metabolism / Q000648:ultrastructure; D006527:Hepatolenticular Degeneration / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D006651:Histocytochemistry; D006801:Humans; D007413:Intestinal Mucosa / Q000378:metabolism / Q000648:ultrastructure*; D007421:Intestine, Small / Q000378:metabolism / Q000648:ultrastructure*; D007583:Jejunum / Q000378:metabolism / Q000648:ultrastructure; D008247:Lysosomes / Q000648:ultrastructure*
D003300:Copper