Abnormalities of the Physiology of Copper in Wilson's Disease

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Abstract
The internal movement of67Cu in the body was studied by serial blood samples and external probe-counting over liver and muscle in seven control subjects, ten homozygotes, and six heterozygotes of Wilson's disease. Plasma clearance was rapid in all subjects, though occasionally delayed in homozygotes. All homozygotes, save one, showed reduced feedback of radioactive copper to plasma, reflecting defective ceruloplasmin biosynthesis. The one exception had elevated serum ceruloplasmin; her plasma radioactive copper curve was normal. All heterozygotes had normalappearing plasma curves. Red blood cell radioactivity was higher than plasma levels after two hours in all homozygotes with abnormal plasma curves. Release of radioactive copper from liver was complete in one week in normals, delayed and incomplete in heterozygotes, and markedly reduced in homozygotes. Muscle uptake was evident in most homozygotes but not in normals or heterozygotes.

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