Biochemical typing of pathological prion protein in aging cattle with BSE.
BACKGROUND
The broad enforcement of active surveillance for bovine spongiform encephalopathy (BSE) in 2000 led to the discovery of previously unnoticed, atypical BSE phenotypes in aged cattle that differed from classical BSE (C-type) in biochemical properties of the pathological prion protein. Depending on the molecular mass and the degree of glycosylation of its proteinase K resistant core fragment (PrPres), mainly determined in samples derived from the medulla oblongata, these atypical cases are currently classified into low (L)-type or high (H)-type BSE. In the present study we address the question to what extent such atypical BSE cases are part of the BSE epidemic in Switzerland.


RESULTS
To this end we analyzed the biochemical PrPres type by Western blot in a total of 33 BSE cases in cattle with a minimum age of eight years, targeting up to ten different brain regions. Our work confirmed H-type BSE in a zebu but classified all other cases as C-type BSE; indicating a very low incidence of H- and L-type BSE in Switzerland. It was documented for the first time that the biochemical PrPres type was consistent across different brain regions of aging animals with C-type and H-type BSE, i.e. independent of the neuroanatomical structure investigated.


CONCLUSIONS
Taken together this study provides further characteristics of the BSE epidemic in Switzerland and generates new baseline data for the definition of C- and H-type BSE phenotypes, thereby underpinning the notion that they indeed represent distinct prion disease entities.
Tester
Seraina
S
NeuroCenter, Reference Laboratory for TSE in animals, Department of Clinical Research and Veterinary Public Health, Vetsuisse Faculty, University of Berne, Switzerland. seraina.tester@itn.unibe.ch
Juillerat
Valerie
V
Doherr
Marcus G
MG
Haase
Bianca
B
Polak
Miroslaw
M
Ehrensperger
Felix
F
Leeb
Tosso
T
Zurbriggen
Andreas
A
Seuberlich
Torsten
T
D000818:Animals; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology* / Q000473:pathology*; D006031:Glycosylation; D017720:Molecular Epidemiology; D008970:Molecular Weight; D015995:Prevalence; D011328:Prions / Q000737:chemistry* / Q000145:classification* / Q000302:isolation & purification; D013557:Switzerland / Q000453:epidemiology
D011328:Prions