Presence of subclinical infection in gene-targeted human prion protein transgenic mice exposed to atypical bovine spongiform encephalopathy

Rona Wilson, Karen Dobie, Nora Hunter, Cristina Casalone, Thierry Baron, Rona Barron

Research output: Contribution to journalArticlepeer-review

Abstract

The transmission of bovine spongiform encephalopathy (BSE) to humans, leading to variant Creutzfeldt-Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health. Until recently, TSE disease in cattle was thought to be caused by a single agent strain, BSE, also known as classical BSE, or BSE-C. However, due to the initiation of a large scale surveillance programme throughout Europe, two atypical BSE strains, bovine amyloidotic spongiform encephalopathy (BASE, also named BSE-L) and BSE-H have since been discovered. To model the risk to human health, we previously inoculated these two forms of atypical BSE (BASE and BSE-H) into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP) (HuTg) but were unable to detect any signs of TSE pathology in these mice. However, despite the absence of TSE pathology, upon subpassage of some BASE challenged HuTg mice, a TSE was observed in recipient gene-targeted bovine PrP Tg (Bov6) mice, but not in HuTg mice. Disease transmission from apparently healthy individuals indicates the presence of subclinical BASE infection in mice expressing human PrP that cannot be identified by current diagnostic methods. However, due to the lack of transmission to HuTg mice on subpassage, the efficiency of mouse to mouse transmission of BASE appears to be low when mice express human rather than bovine PrP.
Original languageEnglish
Pages (from-to)2819-2827
Number of pages9
JournalJournal of General Virology
Volume94
Issue number12
Early online date17 Sep 2013
DOIs
Publication statusPublished - Dec 2013

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