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June 1971

Abnormalities of the Physiology of Copper in Wilson's Disease: II. The Internal Kinetics of Copper

Author Affiliations

San Francisco
From the Department of Neurology, the Division of Clinical Pathology, and the Clinical Study Center, San Francisco General Hospital, San Francisco. Dr. O'Reilly is now with the George Washington University Medical Center, Washington, DC.

Arch Neurol. 1971;24(6):481-488. doi:10.1001/archneur.1971.00480360015001

The internal movement of 67Cu 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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