TY  - JOUR
EP  - 1530
AV  - public
Y1  - 2017/09//
TI  - Protective Effect of Val129-PrP against Bovine Spongiform Encephalopathy but not Variant Creutzfeldt-Jakob Disease
PB  - U.S. National Center for Infectious Diseases
ID  - discovery1568007
N2  - 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.
SP  - 1522
VL  - 23
N1  - This version is the version of record. For information on re-use, please refer to the publisher?s terms and conditions.
IS  - 9
SN  - 1080-6040
UR  - http://doi.org/10.3201/eid2309.161948
A1  - Fernández-Borges, N
A1  - Carlos Espinosa, J
A1  - Marín-Moreno, A
A1  - Aguilar-Calvo, P
A1  - Asante, EAA
A1  - Kitamoto, T
A1  - Mohri, S
A1  - Andréoletti, O
A1  - Torres, JM
JF  - Emerging Infectious Diseases
ER  -