Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate

Autor: Marie-Magdeleine Ruchoux, Emmanuel Comoy, Frédéric Auvré, Jean-Philippe Deslys, Dominique Marcé, Sophie Freire, Nathalie Lescoutra-Etchegaray, Salvatore Monaco, Maria Caramelli, Cristina Casalone, Sergio Ferrari, Corinne Ida Lasmézas, Gianluigi Zanusso, Nicole Salès, Paul Brown, Philippe Leboulch
Jazyk: angličtina
Rok vydání: 2008
Předmět:
Aging
medicine.medical_specialty
Bovine spongiform encephalopathy
animal diseases
prion disease
Encephalopathy
Public Health and Epidemiology/Infectious Diseases
lcsh:Medicine
Disease
Asymptomatic
Macaque
Creutzfeldt-Jakob Syndrome
Bovine Spongiform Encephalopathy
variant Creutzfeldt-Jakob Disease
vCJD
Species Specificity
Biochemistry/Protein Chemistry
Infectious Diseases/Prion Diseases
biology.animal
mental disorders
medicine
Animals
Humans
Genetic Predisposition to Disease
Primate
lcsh:Science
Multidisciplinary
Infectious Diseases/Infectious Diseases of the Nervous System
Virulence
biology
lcsh:R
medicine.disease
Virology
Frontal Lobe
nervous system diseases
Neurological Disorders/Prion Diseases
Encephalopathy
Bovine Spongiform
Macaca fascicularis
Cattle
Histopathology
lcsh:Q
medicine.symptom
Research Article
Zdroj: PLoS ONE, Vol 3, Iss 8, p e3017 (2008)
PLoS ONE
ISSN: 1932-6203
Popis: Background Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains. Methodology/Principal Findings Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine. Conclusion/Significance Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.
Databáze: OpenAIRE
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