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

¹ Departments of Biochemistry and Veterinary Medicine, Mayo Clinic and Mayo Foundation, Rochester 55905; and Department of Small Animal Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota 55108

Copper-laden Bedlington terriers were found to absorb copper normally, based on the ratio of ⁶⁴Cu 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