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Open Access Highly Accessed Research article

The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep

Marion M Simmons1*, Timm Konold1, Lisa Thurston1, Susan J Bellworthy1, Melanie J Chaplin2 and S Jo Moore1

Author Affiliations

1 Department of Pathology, Veterinary Laboratories Agency Weybridge, New Haw, Addlestone KT15 3NB, UK

2 Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency Weybridge, New Haw, Addlestone KT15 3NB, UK

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BMC Veterinary Research 2010, 6:14  doi:10.1186/1746-6148-6-14

Published: 10 March 2010

Abstract

Background

Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries. Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease.

However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. The first successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

Results

This study demonstrates that atypical scrapie has distinct clinical, pathological and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

Conclusions

Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage.