Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein

Wadsworth, Jonathan D.F.; Joiner, Susan; Linehan, Jacqueline M.; Balkema-Buschmann, Anne; Spiropoulos, John; Simmons, Marion M.; Griffiths, Peter C.; Groschup, Martin H.; Hope, James; Brandner, Sebastian; Asante, Emmanuel A.; Collinge, John

doi:10.3201/eid1911.121341

i

Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein

Supporting Files

Nov 2013
By Wadsworth, Jonathan D.F. ; Joiner, Susan ; Linehan, Jacqueline M. ; ...

Details

Alternative Title:

Emerg Infect Dis
Personal Author:

Wadsworth, Jonathan D.F. ; Joiner, Susan ; Linehan, Jacqueline M. ; Balkema-Buschmann, Anne ; Spiropoulos, John ; Simmons, Marion M. ; Griffiths, Peter C. ; Groschup, Martin H. ; Hope, James ; Brandner, Sebastian ; Asante, Emmanuel A. ; Collinge, John
Description:

Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. One example is atypical/Nor98 scrapie, which evaded statutory diagnostic methods worldwide until the early 2000s. To investigate whether sheep infected with scrapie prions could be another source of infection, we inoculated transgenic mice that overexpressed human prion protein with brain tissue from sheep with natural field cases of classical and atypical scrapie, sheep with experimental BSE, and cattle with BSE. We found that these mice were susceptible to BSE prions, but disease did not develop after prolonged postinoculation periods when mice were inoculated with classical or atypical scrapie prions. These data are consistent with the conclusion that prion disease is less likely to develop in humans after exposure to naturally occurring prions of sheep than after exposure to epizootic BSE prions of ruminants.
Subjects:

Atypical Scrapie Bovine Spongiform Encephalopathy BSE Nor98 Scrapie Prion Disease Prion Protein Prions Research Scrapie Sheep BSE Transgenic Mice Variant Creutzfeldt-Jakob Disease VCJD
Source:

Emerg Infect Dis. 19(11):1731-1739.
Document Type:

Journal Article
Funding:

MC_U123160653/Medical Research Council/United Kingdom ; MC_U123160655/Medical Research Council/United Kingdom ; MC_U123192748/Medical Research Council/United Kingdom
Volume:

19
Issue:

11
Collection(s):

Emerging Infectious Diseases
Main Document Checksum:

urn:sha256:c3f68395ce261e73b68630f6583319076309faa5252d4deec75819a4ac4b293a
Download URL:

https://stacks.cdc.gov/view/cdc/20959/cdc_20959_DS1.pdf
File Type:

[PDF - 727.19 KB ]

ON THIS PAGE

Details Supporting Files

CDC STACKS serves as an archival repository of CDC-published products including scientific findings, journal articles, guidelines, recommendations, or other public health information authored or co-authored by CDC or funded partners.

As a repository, CDC STACKS retains documents in their original published format to ensure public access to scientific information.