NR AXDG
AU Richt,J.A.; Kunkle,R.A.; Alt,D.P.; Nicholson,E.M.; Hamir,A.N.; Czub,S.; Kluge,J.; Davis,A.J.; Hall,S.M.
TI Identification and characterization of two bovine spongiform encephalopathy cases diagnosed in the United States
QU Journal of Veterinary Diagnostic Investigation 2007 Mar; 19(2): 142-54
PT case reports; journal article
AB Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the United Kingdom and subsequently in other countries. It is the most likely cause of variant Creutzfeldt-Jakob disease (vCJD) in humans, but the origin of BSE has not been elucidated so far. This report describes the identification and characterization of two cases of BSE diagnosed in the United States. Case 1 (December 2003) exhibited spongiform changes in the obex area of the brainstem and the presence of the abnormal form of the prion protein, PrPsc, in the same brain area, by immunohistochemistry (IHC) and Western blot analysis. Initial suspect diagnosis of BSE for case 2 (November 2004) was made by a rapid ELISA-based BSE test. Case 2 did not exhibit unambiguous spongiform changes in the obex area, but PrPsc was detected by IHC and enrichment Western blot analysis in the obex. Using Western blot analysis, PrPsc from case 1 showed molecular features similar to typical BSE isolates, whereas PrPsc from case 2 revealed an unusual molecular PrPsc pattern: molecular mass of the unglycosylated and monoglycosylated isoform was higher than that of typical BSE isolates and case 2 was strongly labeled with antibody P4, which is consistent with a higher molecular mass. Sequencing of the prion protein gene of both BSE-positive animals revealed that the sequences of both animals were within the range of the prion protein gene sequence diversity previously reported for cattle.
IN Während der erste, im Dezember 2003 in den USA entdeckte BSE-Fall immunhistochemisch und im Western blot den klassischen BSE-Fällen des britischen Typ entsprach, fand man beim im November 2004 per ELISA-Schnelltest identifizierten zweiten US-amerikanischen BSE-Fall keine eindeutigen spongiformen Degenerationen im Obex und im Western blot sah man eine erhöhte scheinbare Molekularmasse des Prionproteins. Nach dieser Darstellung von Richt et al. aus dem Jahr 2007 handelte es sich im zweiten Fall um BSE des H-Typs.
MH Amino Acid Sequence; Animals; Base Sequence; Brain/*pathology; Cattle; DNA/genetics/isolation & purification; Encephalopathy, Bovine Spongiform/*diagnosis/pathology; Goats; Molecular Sequence Data; Polymerase Chain Reaction; Prions/genetics; Sequence Alignment; Sequence Homology, Nucleic Acid; Sheep; United States
AD Jürgen A. Richt (jricht@nadc.ars.usda.gov), Robert A. Kunkle (rkunkle@nadc.ars.usda.gov), David Alt, Eric M. Nicholson, Amir N. Hamir, Stefanie Czub, John Kluge, Arthur J. Davis, S. Mark Hall, Virus and Prion Diseases of Livestock Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, Ames, Iowa 50010, USA
SP englisch
PO USA