AJP - Gastrointestinal and Liver Physiology, Vol 243, Issue 3 226-G230, Copyright © 1982 by American Physiological Society
A comparison of copper-loading disease in Bedlington terriers and Wilson's disease in humans
L. C. Su, S. Ravanshad, C. A. Owen Jr, J. T. McCall, P. E. Zollman and R. M. Hardy
Eleven Bedlington terriers were found to have a mean hepatic copper
concentration of 6,321 micrograms/g dry wt (normal, 200 micrograms/g dry
wt) and renal copper concentration that was three or four times normal.
Brain copper levels were normal in younger dogs, were elevated in two older
dogs, and were 100 times normal in one dog that died of the disease.
Increased concentrations of copper in the liver, kidney, and brain also
characterize Wilson's disease. Erythrocyte survival was normal in three
affected dogs, but serum glutamic-pyruvic transaminase levels were usually
elevated. Unlike the hypoceruloplasminemia of patients with Wilson's
disease, plasma ceruloplasmin activity was not only normal but was also
slightly elevated in the terriers. Despite their normal or excessive
ceruloplasmin, the Bedlington terriers could convert ionic 64Cu to
radioceruloplasmin but did so only very slowly. These dogs accumulated
significantly more 64Cu in their livers than normal, much like patients
with Wilson's disease do before symptoms develop.
