Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie

作者: Neil A. Mabbott , Moira E. Bruce , Marina Botto , Mark J. Walport , Mark B. Pepys

DOI: 10.1038/86562

关键词:

摘要: Following peripheral exposure to transmissible spongiform encephalopathies (TSEs), infectivity usually accumulates in lymphoid tissues before neuroinvasion. The host prion protein (PrPc) is critical for TSE agent replication and as an abnormal, detergent insoluble, relatively proteinase-resistant isoform (PrPSc) diseased tissues1, 2. Early PrPSc accumulation takes place on follicular dendritic cells (FDCs) within germinal centers of patients with variant Creutzfeldt−Jakob disease3 (vCJD), sheep natural scrapie4 or rodents following experimental infection scrapie5, 6, 7. In mouse scrapie models, the absence FDCs blocks spleen, neuroinvasion significantly impaired6, 7, 8, 9. mechanisms by which initially localizes follicles interacts are unknown. Antigens trapped retained surface through interactions between complement cellular receptors10, 11. Here we show that mice, both temporary depletion component C3 genetic deficiency C1q delays onset disease infection, reduces early spleen. Thus, stages perhaps contribute localization tissue may be therapeutic targets.

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