Lysosomal defect of hepatic copper excretion in Wilson's disease (hepatolenticular degeneration).

摘要: After administration of 64Cu, striking differences were revealed in the copper content, specific activity, and protein binding subcellular pools hepatic a woman with Wilson's disease compared control subject. None subject could be identified as source biliary copper, but very low activity 64Cu lysosomes patient was virtually identical to that common duct bile markedly different from all other pools. This suggested might lysosomal defect account for diminution excretion consequent accumulation metal patients disease.