Studies on the Nature and Excretion of Biliary Copper in Man

Abstract

S U M M A R Y 1. Gall-bladder bile was obtained at cholecystectomy from seven patients who had been given [64Cu]cupric acetate intravenously.2. Studies using dialysis and Sephadex gel filtration indicated that most of the 64Cu in the bile was in a heat-stable complex of molecular weight exceeding 50 000. The remaining biliary 64Cu was in a low-molecular-weight component.3. Above pH 4, 64Cu could not readily be removed from the macromolecular complex either by excess carrier copper ions, by D-penicillamine or by EDTA.4. Absorption of 64Cu from 64Cu-labelled bile injected intraduodenally into rats was significantly less than from [64Cu]cupric acetate similarly injected.5. These observations suggest that limitation of an entero-hepatic circulation of copper by a biliary macromolecule which differs from caeruloplasmin, may be a significant factor in copper homeostasis in man.

Liver transplantation for wilson's disease: A single-center experience

“…[1][2][3][4][5][6][22][23][24] This leads to the toxic accumulation of copper in the liver and other organs, such as the brain, kidneys, and corneas. Medical therapy with chelating agents has proven effective in controlling the disease progression and is effective in the prevention of central nervous system complications.…”

“…[26][27][28] Under these circumstances, liver transplantation appears to be the treatment of choice, with correction of the complications of liver failure and acceptable longterm outcomes. Since the first successful liver transplantation for Wilson' s disease in 1969, 29 a number of reports have been accumulated in the literature, addressing the role of transplantation in survival and reversibility of biochemical, 10,19,30 radiological, [20][21][22][23][24][25][26][27][28][29][30][31] and neurological 14,19,32 manifestations of the disease. Nonimmune hemolysis is well Liver Transplantation for Wilson' s Disease described in patients with fulminant Wilson' s disease.…”

Effects of copper source and concentration on copper status and immune function in growing and finishing steers

“…Once dissociated in the stomach, Cu may bind to amino acids or oligopeptides (Allen and Solomons, 1984). Gollan and Dellor (1973) stated that it is likely that low molecular weight substances, such as certain amino acids in gastrointestinal (GI) secretions will bind to Cu and maintain Cu in solution in the upper small intestine. According to Marceau et al (1970), Cu may be bound to certain amino acid complexes before it is absorbed into the gut.…”

Impaired Hepatic Copper Homeostasis in Long-Evans Cinnamon Rats: Reduced Biliary Excretion of Copper

“…They include 1 ) synthesis of abnormal protein with high coppcr binding activity (29). 2 ) persistent fetal mode of copper metabolism (30) on the basis of the concept of a lysosomal defect in hepatocytes, 3) absence or defect ofthe putative carrier proteins in the hepatocyte required for incorporating copper into ceruloplasmin or excreting copper by way of the bile (6,31), and 4) deficiency of copperbinding protein in bile (32)(33)(34), probably the protein identical to serum ceruloplasmin (34). It is also reported that normal human bile contains two molecular forms of ceruloplasmin.…”