Similarities between forms of sheep scrapie and Creutzfeldt-Jakob disease are encoded by distinct prion types.
Transmissible spongiform encephalopathies such as scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and bovine sporadic encephalopathy in cattle are characterized by the accumulation of a misfolded protein: the pathological prion protein. Ever since bovine sporadic encephalopathy was discovered as the likely cause of the new variant of CJD in humans, parallels between human and animal transmissible spongiform encephalopathies must be viewed under the aspect of a disease risk for humans. In our study we have compared prion characteristics of different forms of sheep scrapie with those of different phenotypes of sporadic CJD. The disease characteristics of sporadic CJD depend considerably on the prion type 1 or 2. Our results show that there are obvious parallels between sporadic CJD type 1 and the so-called atypical/Nor98 scrapie. These parelleles apply to the deposition form of pathological prion protein in the brain, detected by the paraffin-embedded-tissue blot and the prion aggregate stability with regard to denaturation by the chaotropic salt guanidine hydrochloride. The same applies to sporadic CJD type 2 and classical scrapie. The observed parallels between types of sporadic CJD and types of sheep scrapie demonstrate that distinct groups of prion disease exist in different species. This should be taken into consideration when discussing interspecies transmission.
Prion and Dementia Research Unit, Department of Neuropathology, University Medical Center Goettingen, Goettingen, Germany.
Wiebke M
WM
Wemheuer
Sylvie L
SL
Benestad
Arne
A
Wrede
Ulf
U
Schulze-Sturm
Wilhelm E
WE
Wemheuer
Uwe
U
Hahmann
Joanna
J
Gawinecka
Ekkehard
E
Schütz
Inga
I
Zerr
Bertram
B
Brenig
Bjørn
B
Bratberg
Olivier
O
Andréoletti
Walter J
WJ
Schulz-Schaeffer
D000818:Animals; D015153:Blotting, Western; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D011328:Prions / Q000737:chemistry* / Q000378:metabolism*; D012608:Scrapie / Q000378:metabolism / Q000473:pathology*; D012756:Sheep
D011328:Prions