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United States Department of Agriculture

Agricultural Research Service


item Richt, Juergen
item Kluge, John
item Alt, David
item Kunkle, Robert
item Hamir, Amirali
item Czub, Stefanie
item Davis, Arthur
item Hall, S Mark

Submitted to: Meeting Abstract
Publication Type: Abstract Only
Publication Acceptance Date: 9/29/2004
Publication Date: 10/14/2004
Citation: Richt, J.A., Kluge, J.P., Alt, D.P., Kunkle, R.A., Hamir, A.N., Czub, S., Davis, A.J., Hall, S.M. 2004. Identification and characterization of the U.S. bovine spongiform encephalopathy case [abstract]. Animal Prion Diseases and the Americas. p. 38.

Interpretive Summary:

Technical Abstract: Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the U.K. and subsequently in other countries. BSE may have arisen from feed contaminated with the scrapie agent, which causes a similar disease in sheep and goats. Here we describe the prion protein polypeptide profile and genotype from the first case of BSE diagnosed in the United States. The animal was found to have spongiform changes in the brainstem area, accompanied by extensive deposition of the abnormal form of the prion protein, PrPres. Brain material was positive by Western blot analysis and an enzyme-linked immunosorbent assay. Comparison of the U.S. BSE isolate to the Canadian BSE isolate and European BSE isolates showed similar sized PrPres polypeptide fragments. The PrP gene from the U.S. BSE case was found to be of bovine origin with a normal cattle PrP sequence. We conclude from these studies that the PrPres profile from the first BSE case diagnosed in the U.S. showed similar molecular properties to the typical PrPres pattern described for the earlier Canadian and European BSE isolates, and that a germline mutation in the bovine PrP gene was not evident.

Last Modified: 09/24/2017
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