Raeber A J, Klein M A, Frigg R, Flechsig E, Aguzzi A, Weissmann C
Institute of Molecular Biology, Department 1, Winterthurerstrasse 190, University of Zürich, Switzerland.
EMBO J. 1999 May 17;18(10):2702-6. doi: 10.1093/emboj/18.10.2702.
An intact immune system, and particularly the presence of mature B lymphocytes, is crucial for mouse scrapie pathogenesis in the brain after peripheral exposure. Prions are accumulated in the lymphoreticular system (LRS), but the identity of the cells containing infectivity and their role in neuroinvasion have not been determined. We show here that although prion infectivity in the spleen is associated with B and T lymphocytes and to a lesser degree with the stroma, no infectivity could be detected in lymphocytes from blood. In wild-type mice, which had been irradiated and reconstituted with PrP-deficient lymphohaematopoietic stem cells and inoculated with scrapie prions, infectivity in the spleen was present in the stroma but not in lymphocytes. Therefore, splenic B and T lymphocytes can either synthesize prions or acquire them from another source, but only when they express PrP.
完整的免疫系统，尤其是成熟B淋巴细胞的存在，对于外周暴露后小鼠脑中痒病的发病机制至关重要。朊病毒在淋巴网状系统（LRS）中积累，但含有传染性的细胞的身份及其在神经侵袭中的作用尚未确定。我们在此表明，虽然脾脏中的朊病毒传染性与B淋巴细胞和T淋巴细胞相关，且在较小程度上与基质相关，但在血液淋巴细胞中未检测到传染性。在用PrP缺陷的淋巴造血干细胞进行辐照和重建并接种痒病朊病毒的野生型小鼠中，脾脏中的传染性存在于基质中而非淋巴细胞中。因此，脾脏B淋巴细胞和T淋巴细胞要么可以合成朊病毒，要么从另一个来源获取朊病毒，但前提是它们表达PrP。
