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- Title
Protective Effect of Val<sub>129</sub>-PrP against Bovine Spongiform Encephalopathy but not Variant Creutzfeldt-Jakob Disease.
- Authors
Fernández-Borges, Natalia; Espinosa, Juan Carlos; Marín-Moreno, Alba; Aguilar-Calvo, Patricia; Asante, Emmanuel A.; Tetsuyuki Kitamoto; Shirou Mohri; Andréoletti, Olivier; Torres, Juan María; Kitamoto, Tetsuyuki; Mohri, Shirou
- Abstract
Bovine spongiform encephalopathy (BSE) is the only known zoonotic prion that causes variant Creutzfeldt-Jakob disease (vCJD) in humans. The major risk determinant for this disease is the polymorphic codon 129 of the human prion protein (Hu-PrP), where either methionine (Met129) or valine (Val129) can be encoded. To date, all clinical and neuropathologically confirmed vCJD cases have been Met129 homozygous, with the exception of 1 recently reported Met/Val heterozygous case. Here, we found that transgenic mice homozygous for Val129 Hu-PrP show severely restricted propagation of the BSE prion strain, but this constraint can be partially overcome by adaptation of the BSE agent to the Met129 Hu-PrP. In addition, the transmission of vCJD to transgenic mice homozygous for Val129 Hu-PrP resulted in a prion with distinct strain features. These observations may indicate increased risk for vCJD secondary transmission in Val129 Hu-PrP-positive humans with the emergence of new strain features.
- Subjects
BOVINE spongiform encephalopathy; CREUTZFELDT-Jakob disease; VALINE; GENETIC polymorphisms; PRIONS; TRANSGENIC mice; METHIONINE; DISEASE risk factors; THERAPEUTICS; AMINO acids; ANIMAL experimentation; BRAIN; CATTLE; COMPARATIVE studies; GENE expression; GENES; RESEARCH methodology; MEDICAL cooperation; MICE; NATURAL immunity; PROTEOLYTIC enzymes; RESEARCH; RESEARCH funding; EVALUATION research; INTRAVENTRICULAR injections; INFECTIOUS disease transmission
- Publication
Emerging Infectious Diseases, 2017, Vol 23, Issue 9, p1522
- ISSN
1080-6040
- Publication type
journal article
- DOI
10.3201/eid2309.161948