A copper-sulfur complex in the liver of a patient with Wilson's disease.
An asymptomatic 16-year-old boy was found to have Wilson's disease without Kayser-Fleischer rings. Liver biopsy showed chronic active hepatitis with 1025 micrograms copper/g dry weight. After 19 months of d-penicillamine therapy, the liver histology became almost normal and the copper content decreased to 238 micrograms/g dry weight. The liver specimens obtained before and after treatment were studied by X-ray probe microanalysis. After treatment, both copper and sulfur decreased in hepatocellular lysosomes. The estimated molar ratio of the decreased copper to the decreased sulfur was 32/100. These figures suggest that lysosomal copper exists in the form of metallothionein.
D000293:Adolescent; D001706:Biopsy; D002118:Calcium / Q000378:metabolism; D003300:Copper / Q000378:metabolism*; D004577:Electron Probe Microanalysis; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008658:Inactivation, Metabolic; D007501:Iron / Q000378:metabolism; D008247:Lysosomes / Q000378:metabolism; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D010758:Phosphorus / Q000378:metabolism; D013455:Sulfur / Q000378:metabolism*
D010758:Phosphorus; D013455:Sulfur; D003300:Copper; D007501:Iron; D010396:Penicillamine; D002118:Calcium