Protective Effect of Val129-PrP against Bovine Spongiform Encephalopathy but not Variant Creutzfeldt-Jakob Disease

被引：27

作者：

Fernandez-Borges, Natalia ^{[1
]}

Carlos Espinosa, Juan ^{[1
]}

Marin-Moreno, Alba ^{[1
]}

Aguilar-Calvo, Patricia ^{[1
]}

Asante, Emmanuel A. ^{[2
]}

Kitamoto, Tetsuyuki ^{[3
]}

Mohri, Shirou ^{[3
]}

Andreoletti, Olivier ^{[4
]}

Maria Torres, Juan ^{[1
]}

机构：

[1] Inst Nacl Invest & Tecnol Agr & Alimentaria CISA, Ctr Invest Sanidad Anim, Madrid, Spain

[2] UCL, Inst Neurol, MRC Prion Unit, Dept Neurodegenerat Dis, London, England

[3] Tohoku Univ, Grad Sch Med, Sendai, Miyagi, Japan

[4] Ecole Natl Vet Toulouse, Interact Hotes Agents Pathogenes, UMR INRA ENVT 1225, Toulouse, France

来源：

EMERGING INFECTIOUS DISEASES | 2017年 / 23卷 / 09期

关键词：

HUMAN PRION PROTEIN; TRANSGENIC MICE; BLOOD-TRANSFUSION; MOLECULAR-BASIS; TISSUE SAMPLES; BSE AGENT; SCRAPIE; CJD; TRANSMISSION; SHEEP;

D O I：

10.3201/eid2309.161948

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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 (Met(129)) or valine (Val(129)) can be encoded. To date, all clinical and neuropathologically confirmed vCJD cases have been Met(129) homozygous, with the exception of 1 recently reported Met/Val heterozygous case. Here, we found that transgenic mice homozygous for Val(129) 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 Met(129) Hu-PrP. In addition, the transmission of vCJD to transgenic mice homozygous for Val(129) Hu-PrP resulted in a prion with distinct strain features. These observations may indicate increased risk for vCJD secondary transmission in Val(129) Hu-PrP-positive humans with the emergence of new strain features.

引用

页码：1522 / 1530

页数：9

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