UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076, Toulouse, France.
APHA Sutton Bonington, Loughborough, LE12 5NB, Leicestershire, UK.
Acta Neuropathol. 2021 Mar;141(3):383-397. doi: 10.1007/s00401-021-02270-x. Epub 2021 Feb 2.
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 PrP 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.
散发性 Creutzfeldt-Jakob 病（sCJD）是最常见的人类朊病毒病，很可能是由于中枢神经系统（CNS）中异常朊病毒蛋白的自发形成所致。变异型 Creutzfeldt-Jakob 病（vCJD）是一种获得性朊病毒病，于 1996 年首次被发现。与 vCJD 形成鲜明对比的是，以前对 sCJD 的研究显示，在外周组织中，PrP 的水平要么不一致，要么不存在。这些发现促使人们达成共识，即由于非 CNS 侵袭性临床操作而导致 sCJD 传播的风险较低。在这项研究中，我们系统地测量了来自 vCJD 和 sCJD 患者的中枢神经系统和外周组织中的朊病毒感染性水平。出乎意料的是，在 sCJD 病例的各种外周组织中检测到了朊病毒感染性。尽管在采样的组织中和患者之间，sCJD 的感染性水平变化不可预测，但这些发现可能会影响我们对与 sCJD 相关的可能传播风险的认识。
