Key result
Experimental transmission and sub-passage of atypical scrapie in AHQ homozygous sheep consistently preserved its distinct clinical, pathological, and biochemical phenotype.
Atypical scrapie is consistently transmissible within AHQ homozygous sheep with preservation of its distinct disease phenotype on sub-passage.
May indicate experimental transmissibility of atypical scrapie; leaves open spontaneous origin in natural settings.
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.
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Simmons et al. (2010) studied Atypical scrapie (n=20). Intracerebral inoculation with atypical scrapie vs. Classical scrapie and BSE inoculations was evaluated on Preservation of atypical scrapie phenotype (clinical, pathological, biochemical). Experimental transmission and sub-passage of atypical scrapie in AHQ homozygous sheep consistently preserved its distinct clinical, pathological, and biochemical phenotype.
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