» Articles » PMID: 21347349

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

Abstract

Atypical/Nor98 scrapie was first identified in 1998 in Norway. It is now considered as a worldwide disease of small ruminants and currently represents a significant part of the detected transmissible spongiform encephalopathies (TSE) cases in Europe. Atypical/Nor98 scrapie cases were reported in ARR/ARR sheep, which are highly resistant to BSE and other small ruminants TSE agents. The biology and pathogenesis of the Atypical/Nor98 scrapie agent in its natural host is still poorly understood. However, based on the absence of detectable abnormal PrP in peripheral tissues of affected individuals, human and animal exposure risk to this specific TSE agent has been considered low. In this study we demonstrate that infectivity can accumulate, even if no abnormal PrP is detectable, in lymphoid tissues, nerves, and muscles from natural and/or experimental Atypical/Nor98 scrapie cases. Evidence is provided that, in comparison to other TSE agents, samples containing Atypical/Nor98 scrapie infectivity could remain PrP(Sc) negative. This feature will impact detection of Atypical/Nor98 scrapie cases in the field, and highlights the need to review current evaluations of the disease prevalence and potential transmissibility. Finally, an estimate is made of the infectivity loads accumulating in peripheral tissues in both Atypical/Nor98 and classical scrapie cases that currently enter the food chain. The results obtained indicate that dietary exposure risk to small ruminants TSE agents may be higher than commonly believed.

Citing Articles

Prions in Muscles of Cervids with Chronic Wasting Disease, Norway.

Vuong T, Cazzaniga F, Tran L, Vage J, Di Bari M, Pirisinu L Emerg Infect Dis. 2025; 31(2):246-255.

PMID: 39983705 PMC: 11845164. DOI: 10.3201/eid3102.240903.


Strain-Specific Targeting and Destruction of Cells by Prions.

Simmons S, Bartz J Biology (Basel). 2024; 13(1).

PMID: 38275733 PMC: 10813089. DOI: 10.3390/biology13010057.


Diagnosis in Scrapie: Conventional Methods and New Biomarkers.

Sola D, Betancor M, Marco Lorente P, Perez Lazaro S, Barrio T, Sevilla E Pathogens. 2023; 12(12).

PMID: 38133284 PMC: 10746075. DOI: 10.3390/pathogens12121399.


Heterogeneity of pathological prion protein accumulation in the brain of moose (Alces alces) from Norway, Sweden and Finland with chronic wasting disease.

Sola D, Tran L, Vage J, Madslien K, Vuong T, Korpenfelt S Vet Res. 2023; 54(1):74.

PMID: 37684668 PMC: 10492377. DOI: 10.1186/s13567-023-01208-3.


An arrayed genome-wide perturbation screen identifies the ribonucleoprotein Hnrnpk as rate-limiting for prion propagation.

Avar M, Heinzer D, Thackray A, Liu Y, Hruska-Plochan M, Sellitto S EMBO J. 2022; 41(23):e112338.

PMID: 36254605 PMC: 9713719. DOI: 10.15252/embj.2022112338.


References
1.
Race R, Raines A, Raymond G, Caughey B, Chesebro B . Long-term subclinical carrier state precedes scrapie replication and adaptation in a resistant species: analogies to bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease in humans. J Virol. 2001; 75(21):10106-12. PMC: 114584. DOI: 10.1128/JVI.75.21.10106-10112.2001. View

2.
Fediaevsky A, Morignat E, Ducrot C, Calavas D . A case-control study on the origin of atypical scrapie in sheep, France. Emerg Infect Dis. 2009; 15(5):710-8. PMC: 2687017. DOI: 10.3201/eid1505.081119. View

3.
Luhken G, Buschmann A, Brandt H, Eiden M, Groschup M, Erhardt G . Epidemiological and genetical differences between classical and atypical scrapie cases. Vet Res. 2006; 38(1):65-80. DOI: 10.1051/vetres:2006046. View

4.
Tixador P, Herzog L, Reine F, Jaumain E, Chapuis J, Le Dur A . The physical relationship between infectivity and prion protein aggregates is strain-dependent. PLoS Pathog. 2010; 6(4):e1000859. PMC: 2855332. DOI: 10.1371/journal.ppat.1000859. View

5.
Nentwig A, Oevermann A, Heim D, Botteron C, Zellweger K, Drogemuller C . Diversity in neuroanatomical distribution of abnormal prion protein in atypical scrapie. PLoS Pathog. 2007; 3(6):e82. PMC: 1891327. DOI: 10.1371/journal.ppat.0030082. View