期刊
ACTA NEUROPATHOLOGICA
卷 141, 期 3, 页码 383-397出版社
SPRINGER
DOI: 10.1007/s00401-021-02270-x
关键词
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资金
- 'Fond Europeen de Developpement Regional, Programme Operationnel de Cooperation Territoriale Espagne France Andorre' REDPRION [EFA148/16]
- UK Food Standards Agency Exploring permeability of the species barrier [M03043, FS231051]
- European Union [222887]
- Department of Health
- Scottish Government
- Medical Research Council
Sporadic Creutzfeldt-Jakob disease (sCJD) typically occurs in the central nervous system, but recent research has shown prion infectivity detected in peripheral tissues of patients as well. Although infectivity levels varied unpredictably among different patients and tissues, these findings may alter our perception of transmission risks associated with sCJD.
Sporadic Creutzfeldt-Jakob disease (sCJD) is the commonest human prion disease, occurring most likely as the consequence of spontaneous formation of abnormal prion protein in the central nervous system (CNS). Variant Creutzfeldt-Jakob disease (vCJD) is an acquired prion disease that was first identified in 1996. In marked contrast to vCJD, previous investigations in sCJD revealed either inconsistent levels or an absence of PrPSc in peripheral tissues. These findings contributed to the consensus that risks of transmitting sCJD as a consequence of non-CNS invasive clinical procedures were low. In this study, we systematically measured prion infectivity levels in CNS and peripheral tissues collected from vCJD and sCJD patients. Unexpectedly, prion infectivity was detected in a wide variety of peripheral tissues in sCJD cases. Although the sCJD infectivity levels varied unpredictably in the tissues sampled and between patients, these findings could impact on our perception of the possible transmission risks associated with sCJD.
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