The Subcellular Distribution of Liver Copper in Normal Subjects and Patients with Primary Biliary Cirrhosis and Wilson's Disease

1980 ◽  
Vol 58 (2) ◽  
pp. 14P-14P
Author(s):  
W. J. Jenkins ◽  
J. Evans ◽  
O. Epstein
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Subcellular Distribution ◽  
Wilson’S Disease ◽  
Normal Subjects ◽  
Wilson's Disease ◽  
Biliary Cirrhosis ◽  
Liver Copper

Studies with Radioactive Copper (64Cu and 67Cu); The Incorporation of Radioactive Copper into Caeruloplasmin in Wilson's Disease and in Primary Biliary Cirrhosis

1971 ◽  
Vol 41 (3) ◽  
pp. 189-202 ◽  
Author(s):  
Kathleen Gibbs ◽  
J. M. Walshe
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Control Subject ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Turnover Time ◽  
Control Group ◽  
Biliary Cirrhosis ◽  
Liver Copper ◽  
Secondary Rise ◽  
Copper Overload

1. A comparison has been made of the handling of radioactive copper by patients with Wilson's disease, patients with primary biliary cirrhosis and a small control group. The object of this study has been to assess the influence of an expanded hepatic pool of copper on the incorporation of radioactive copper into caeruloplasmin. 2. Studies with 67Cu have shown that radioactivity disappears steadily from the plasma of patients with Wilson's disease for periods up to 296 h. Both in patients with primary biliary cirrhosis and in controls, a well-marked secondary rise occurs as radioactive copper is incorporated into caeruloplasmin. 3. Caeruloplasmin has been isolated from the serum of one control subject, one patient with primary biliary cirrhosis and one with Wilson's disease. Of the plasma radioactivity 90% was present in the caeruloplasmin of the control subject and the patient with primary biliary cirrhosis; only 27% was found in the caeruloplasmin of the patient with Wilson's disease. 4. Turnover time for liver copper has been determined; in the control subjects it was of the order of 20–30 days, in the patients with primary biliary cirrhosis it was between 600 and 700 days, in Wilson's disease the time exceeded 1800 days. In all cases the turnover time for radioactive copper was significantly less. 5. We conclude (a) that newly arrived radioactive copper is preferentially handled in the presence of copper overload and (b) dilution of radioactive copper in an expanded hepatic pool of the metal cannot alone account for the delayed incorporation found in patients with Wilson's disease.

Incorporation of radiocopper into ceruloplasmin in normal subjects and in patients with primary biliary cirrhosis and Wilson's disease

1978 ◽  
Vol 74 (4) ◽  
pp. 652-660 ◽  
Author(s):  
John M. Vierling ◽  
Richard Shrager ◽  
Warren F. Rumble ◽  
Roger Aamodt ◽  
Marvin D. Berman ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Wilson’S Disease ◽  
Normal Subjects ◽  
Wilson's Disease ◽  
Biliary Cirrhosis

scholarly journals A Patient With Primary Biliary Cirrhosis Accompanied by Wilson’s Disease

2016 ◽  
Vol 16 (2) ◽  
Author(s):  
Su-Xian Zhao ◽  
Yu-Guo Zhang ◽  
Rong-Qi Wang ◽  
Wen-Cong Li ◽  
Ling-Bo Kong ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Biliary Cirrhosis

The ontogeny of liver copper metabolism in the guinea pig: Clues to the etiology of Wilson's disease

Hepatology ◽  
1986 ◽  
Vol 6 (3) ◽  
pp. 427-432 ◽  
Author(s):  
Surjit K. S. Srai ◽  
Andrew K. Burroughs ◽  
Bernard Wood ◽  
Owen Epstein
Keyword(s):  
Guinea Pig ◽  
Wilson’S Disease ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Liver Copper

Absorption of Copper in Homozygotes and Heterozygotes for Wilson's Disease and Controls: Isotope Tracer Studies with 67Cu and 64Cu

1972 ◽  
Vol 43 (5) ◽  
pp. 617-625 ◽  
Author(s):  
G. T. Strickland ◽  
W. M. Beckner ◽  
Mei-Ling Leu
Keyword(s):  
Wilson’S Disease ◽  
Body Burden ◽  
Normal Subjects ◽  
Wilson's Disease ◽  
Cirrhosis Of The Liver ◽  
Whole Body ◽  
Faecal Excretion ◽  
Body Retention ◽  
Body Counting ◽  
The Mean

1. Absorption of copper was determined by the simultaneous administration of 64Cu orally and 67Cu intravenously to six patients with Wilson's disease (WD), eighteen of their parents and siblings, four normal subjects and three subjects with cirrhosis of the liver. Absorption was calculated by three methods: (1) the mean ratio of 64Cu to 67Cu body retention at 3 and 4 days as determined by whole-body counting; (2) the mean ratio of 64Cu to 67Cu at 3 and 4 days as determined by faecal excretion; and (3) the mean ratio of 64Cu to 67Cu plasma radioactivity 6–24 h after administration. 2. The total-body counting and faecal methods for determining copper absorption agreed with each other, demonstrating that the normal absorption of copper is 40–70% (mean 56%) of the dose and that absorption is not influenced by cirrhosis of the liver, age or sex; but it appears to be inversely related to the amount of carrier copper. The absorption of copper in both homozygotes and heterozygotes for WD did not differ significantly from that of the control subjects. Therefore, the increased body burden of copper in WD does not appear to be due to over absorption, but rather to decreased biliary excretion of copper.

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