Intraspecies transmission of BASE induces clinical dullness and amyotrophic changes.

Abstract:

:The disease phenotype of bovine spongiform encephalopathy (BSE) and the molecular/ biological properties of its prion strain, including the host range and the characteristics of BSE-related disorders, have been extensively studied since its discovery in 1986. In recent years, systematic testing of the brains of cattle coming to slaughter resulted in the identification of at least two atypical forms of BSE. These emerging disorders are characterized by novel conformers of the bovine pathological prion protein (PrP(TSE)), named high-type (BSE-H) and low-type (BSE-L). We recently reported two Italian atypical cases with a PrP(TSE) type identical to BSE-L, pathologically characterized by PrP amyloid plaques and known as bovine amyloidotic spongiform encephalopathy (BASE). Several lines of evidence suggest that BASE is highly virulent and easily transmissible to a wide host range. Experimental transmission to transgenic mice overexpressing bovine PrP (Tgbov XV) suggested that BASE is caused by a prion strain distinct from the BSE isolate. In the present study, we experimentally infected Friesian and Alpine brown cattle with Italian BSE and BASE isolates via the intracerebral route. BASE-infected cattle developed amyotrophic changes accompanied by mental dullness. The molecular and neuropathological profiles, including PrP deposition pattern, closely matched those observed in the original cases. This study provides clear evidence of BASE as a distinct prion isolate and discloses a novel disease phenotype in cattle.

journal_name

PLoS Pathog

journal_title

PLoS pathogens

authors

Lombardi G,Casalone C,D' Angelo A,Gelmetti D,Torcoli G,Barbieri I,Corona C,Fasoli E,Farinazzo A,Fiorini M,Gelati M,Iulini B,Tagliavini F,Ferrari S,Caramelli M,Monaco S,Capucci L,Zanusso G

doi

10.1371/journal.ppat.1000075

subject

Has Abstract

pub_date

2008-05-23 00:00:00

pages

e1000075

issue

5

eissn

1553-7366

issn

1553-7374

journal_volume

4

pub_type

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