CEA, Institut François Jacob, Université Paris-Saclay, 18 Route du Panorama, 92265, Fontenay-aux-Roses, France.
MacoPharma, 200 Chaussée Fernand Forest, 59200, Tourcoing, France.
Nat Commun. 2017 Nov 2;8(1):1268. doi: 10.1038/s41467-017-01347-0.
Exposure of human populations to bovine spongiform encephalopathy through contaminated food has resulted in <250 cases of variant Creutzfeldt-Jakob disease (vCJD). However, more than 99% of vCJD infections could have remained silent suggesting a long-term risk of secondary transmission particularly through blood. Here, we present experimental evidence that transfusion in mice and non-human primates of blood products from symptomatic and non-symptomatic infected donors induces not only vCJD, but also a different class of neurological impairments. These impairments can all be retransmitted to mice with a pathognomonic accumulation of abnormal prion protein, thus expanding the spectrum of known prion diseases. Our findings suggest that the intravenous route promotes propagation of masked prion variants according to different mechanisms involved in peripheral replication.
人类通过受污染的食物接触牛海绵状脑病，导致<250 例变异型克雅氏病（vCJD）。然而，超过 99%的 vCJD 感染可能一直处于潜伏状态，这表明特别是通过血液存在长期的二次传播风险。在这里，我们提供了实验证据，表明在小鼠和非人类灵长类动物中输注来自有症状和无症状感染供体的血液制品，不仅会引起 vCJD，还会引起不同类型的神经损伤。这些损伤都可以通过异常朊病毒蛋白的特征性积累重新传播到小鼠中，从而扩大了已知朊病毒疾病的范围。我们的发现表明，静脉途径根据外周复制中涉及的不同机制促进了隐匿朊病毒变异体的传播。
