A defect of biliary excretion of copper in copper-laden Bedlington terriers

Copper-laden Bedlington terriers were found to absorb copper normally, based on the ratio of 64 Cu in bile and in plasma ceruloplasmin after oral and intravenous doses of the radionuclide. The excessive accumulation of the copper in Bedlington terriers was due to impaired biliary excretion of the metal, The abnormal Bedlington pattern was simulated in normal dogs by biliary obstruction. Although the Bedlington terriers excreted more copper in their urine than normal, the amount was far less than that accumulated secondary to inadequate biliary excretion. These abnormalities are shared by patients with Wilson's disease, both conditions being inherited on an autosomal recessive basis. copper absorption; copper excretion; radiocopper; Wilson's disease Submitted on May 5, 1981 Accepted on March 30, 1982