Conversion of the BASE Prion Strain into the BSE Strain: The Origin of BSE?

PLoS Pathogens

Gianluigi Zanusso¹  Salvatore Monaco¹  Giuseppe Di Fede²  Lucia Limido²  Ludovico Minati²  Maria Grazia Bruzzone²  Giorgio Giaccone²  Fabrizio Tagliavini²  Marcella Catania²  Raffaella Capobianco²  Silvia Suardi²  Michela Mangieri²  Giacomina Rossi²  Claudia Miccolo²  Anne Buschmann³  Martin H Groschup³  Cristiano Corona⁴  Pierluigi Acutis⁴  Maria Caramelli⁴  Cristina Casalone⁴  Daniela Gelmetti⁵  Guerino Lombardi⁵ 

[1] Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G. B. Rossi, Verona, Italy;Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta, Milan, Italy;Friedrich-Loeffler-Institut, Institute for Novel and Emerging Infectious Diseases, Greifswald, Insel Riems, Germany;Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Torino, Italy;Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Brescia, Italy

关键词: Bovine spongiform encephalopathy;    Animal prion diseases;    Cattle;    Inbreeding;    Inbred strains;    Veterinary diseases;    Mouse models;    Creutzfeldt-Jakob disease;   

DOI  :  10.1371/journal.ppat.0030031

学科分类：生物科学（综合）

来源: Public Library of Science

【 摘 要 】

Atypical neuropathological and molecular phenotypes of bovine spongiform encephalopathy (BSE) have recently been identified in different countries. One of these phenotypes, named bovine “amyloidotic” spongiform encephalopathy (BASE), differs from classical BSE for the occurrence of a distinct type of the disease-associated prion protein (PrP), termed PrPSc, and the presence of PrP amyloid plaques. Here, we show that the agents responsible for BSE and BASE possess different biological properties upon transmission to transgenic mice expressing bovine PrP and inbred lines of nontransgenic mice. Strikingly, serial passages of the BASE strain to nontransgenic mice induced a neuropathological and molecular disease phenotype indistinguishable from that of BSE-infected mice. The existence of more than one agent associated with prion disease in cattle and the ability of the BASE strain to convert into the BSE strain may have important implications with respect to the origin of BSE and spongiform encephalopathies in other species, including humans.

【 授权许可】

CC BY