title: Detection and discrimination of classical and atypical L-type bovine spongiform encephalopathy by real-time quaking-induced conversion.
abstract: Statutory surveillance of bovine spongiform encephalopathy (BSE) indicates that cattle are susceptible to both classical BSE (C-BSE) and atypical forms of BSE. Atypical forms of BSE appear to be sporadic and thus may never be eradicated. A major challenge for prion surveillance is the lack of sufficiently practical and sensitive tests for routine BSE detection and strain discrimination. The real-time quaking-induced conversion (RT-QuIC) test, which is based on prion-seeded fibrillization of recombinant prion protein (rPrPSen), is known to be highly specific and sensitive for the detection of multiple human and animal prion diseases but not BSE. Here, we tested brain tissue from cattle affected by C-BSE and atypical L-type bovine spongiform encephalopathy (L-type BSE or L-BSE) with the RT-QuIC assay and found that both BSE forms can be detected and distinguished using particular rPrPSen substrates. Specifically, L-BSE was detected using multiple rPrPSen substrates, while C-BSE was much more selective. This substrate-based approach suggests a diagnostic strategy for specific, sensitive, and rapid detection and discrimination of at least some BSE forms.
authors.affiliation: Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana, USA.
authors.forename: Christina D
authors.initials: CD
authors.lastname: Orrú
authors.affiliation: National Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Favole
authors.affiliation: National Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Cristiano
authors.initials: C
authors.lastname: Corona
authors.affiliation: National Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Mazza
authors.affiliation: Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana, USA.
authors.forename: Matteo
authors.initials: M
authors.lastname: Manca
authors.affiliation: Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana, USA.
authors.forename: Bradley R
authors.initials: BR
authors.lastname: Groveman
authors.affiliation: Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana, USA.
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Hughson
authors.affiliation: National Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Acutis
authors.affiliation: National Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Caramelli
authors.affiliation: Department of Neurological and Movement Sciences, University of Verona, Verona, Italy.
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Zanusso
authors.affiliation: National Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy cristina.casalone@izsto.it bcaughey@nih.gov.
authors.forename: Cristina
authors.initials: C
authors.lastname: Casalone
authors.affiliation: Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana, USA cristina.casalone@izsto.it bcaughey@nih.gov.
authors.forename: Byron
authors.initials: B
authors.lastname: Caughey
mesh_terms: D000818:Animals; D001681:Biological Assay / Q000379:methods*; D001923:Brain Chemistry; D002417:Cattle; D006224:Cricetinae; D016643:Encephalopathy, Bovine Spongiform / Q000145:classification* / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D011328:Prions / Q000032:analysis* / Q000737:chemistry* / Q000378:metabolism; D011994:Recombinant Proteins
chemical_list: D011328:Prions; D011994:Recombinant Proteins