Institute of Neurology, Medical University of Vienna, Vienna, Austria.
Austrian Reference Center for Human Prion Diseases, Medical University of Vienna, Vienna, Austria.
Ann Neurol. 2019 May;85(5):782-787. doi: 10.1002/ana.25451. Epub 2019 Mar 11.
Disease-associated proteins are thought to propagate along neuronal processes in neurodegenerative diseases. To detect disease-associated prion protein (PrP ) in the vagus nerve in different forms and molecular subtypes of Creutzfeldt-Jakob disease (CJD), we applied 3 different anti-PrP antibodies. We screened the vagus nerve in 162 sporadic and 30 genetic CJD cases. Four of 31 VV-2 type sporadic CJD and 7 of 30 genetic CJD cases showed vagal PrP immunodeposits with distinct morphology. Thus, PrP in CJD affects the vagus nerve analogously to α-synuclein in Parkinson disease. The morphologically diverse deposition of PrP in genetic and sporadic CJD argues against uniform mechanisms of propagation of PrP . Ann Neurol 2019;85:782-787.
在神经退行性疾病中，疾病相关蛋白被认为沿着神经元过程传播。为了在不同形式和分子亚型的克雅氏病（CJD）中检测迷走神经中的疾病相关朊病毒蛋白（PrP），我们应用了 3 种不同的抗 PrP 抗体。我们对 162 例散发性和 30 例遗传性 CJD 病例的迷走神经进行了筛选。4 例 VV-2 型散发性 CJD 和 30 例遗传性 CJD 中有 7 例出现了具有独特形态的迷走神经 PrP 免疫沉积物。因此，CJD 中的 PrP 与帕金森病中的 α-突触核蛋白类似地影响迷走神经。遗传和散发性 CJD 中 PrP 的形态多样的沉积表明 PrP 的传播机制并不统一。神经病学杂志 2019；85:782-787。
