Bedlington Terriers Research Articles

Inherited copper toxicosis in Bedlington terriers

Australian Veterinary JournalVolume 59, Issue 4 p. 128-128 Inherited copper toxicosis in Bedlington terriers V. P. STUDDERT, V. P. STUDDERT Veterinary Clinical Centre, University of Melbourne, Werribee, Victoria, 3030Search for more papers by this author V. P. STUDDERT, V. P. STUDDERT Veterinary Clinical Centre, University of Melbourne, Werribee, Victoria, 3030Search for more papers by this author First published: October 1982 https://doi.org/10.1111/j.1751-0813.1982.tb02753.xCitations: 1AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume59, Issue4October 1982Pages 128-128 RelatedInformation

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 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

Hepatic lysosomal copper protein in dogs with an inherited copper toxicosis.

Hepatic copper overload inherited as an autosomal recessive trait in Bedlington Terriers is characterized by the presence of hepatocellular lysosomal granules of unusually high specific gravity and electron density which contain at least two thirds of the total hepatic copper. Fractionation of homogenates of liver from such affected Bedlington Terriers yielded a low-speed pellet which contained the lysosomal granules. This fraction was used for isolation of a copper-binding protein by alkaline-reduction, solubilization, fractional acetone precipitation, and gel filtration. The purified protein yielded a single band on polyacrylamide gel electrophoresis and resembled other metallothioneins in containing 15 cysteine residues and 7 to 8 atoms of copper (but no zinc) per 54 amino acid residues. No methionine, histidine, or any aromatic amino acid was present. The accumulation of this lysosomal copper protein appears to be related to the primary genetic defect which underlies the hepatic copper toxicosis of the Bedlington Terrier.

Copper and hepatic function.

When hepatic excretion of copper into bile is impaired, the amount of copper in the liver increases. This happens in extrahepatic cholestasis, primary biliary cirrhosis and in two inherited diseases of copper disease. By six months of age the homozygously affected Bedlington terrier has already begun to accumulate copper in its liver. The trend continues, peaking at the age of five to eight years, when hepatic copper may exceed 10 000 microgram/g dry weight (normal 90--400 microgram/g in livers from mongrels). Despite these concentrations, which are several times higher than those found in any human disease, there is remarkable little evidence of hepatic inflammation or fibrosis in younger Bedlington terriers. The copper is condensed in lysosomes and is identified by X-ray emission spectroscopy. Hepatic cirrhosis eventually develops and death is often associated with ascites and jaundice. Despite characteristic histological differences between the livers in Wilson's disease and in the Bedlington disease, there is a striking general resemblance between the two conditions.

Hemostasis in the copper-laden Bedlington terrier: a possible model of Wilson's disease.

A hemostatic survey was done on 14 Bedlington terriers, 13 of which have the recently discovered copper toxicosis. Their hepatic copper ranged from 109 to 9,888 microgram/g dry weight and their ages from 8 months to 8 years. Despite histologic evidence of hepatitis in younger dogs and cirrhosis in older ones, plasmatic coagulation factors were not depressed. In fact, the hemophilic factors VIII, IX and XI were above normal, more closely related to the age of the dog than to the hepatic copper. Furthermore, their platelet were unusually sensitive to adenosine diphosphate exposure. Offsprings of matings between Bedlington terriers and Beagles seem to be normal.

Fatal disease in bedlington terriers

Fatal disease in bedlington terriers

Inherited Copper Toxicity of the Liver in Bedlington Terriers

Inherited Copper Toxicity of the Liver in Bedlington Terriers