Division of Patient Based Clinical Research, Toronto Western Research Institute, and the Edmond J, Safra Program in Parkinson's Disease, Toronto Western Hospital, McLaughlin Pavilion, 7th Floor Rm 7-403, 399 Bathurst Street, Toronto, Ontario M5T 2S8, Canada.
Acta Neuropathol Commun. 2013 May 8;1:2. doi: 10.1186/2051-5960-1-2.
Parkinson's disease (PD) is a progressive neurodegenerative disorder typified by the presence of intraneuronal inclusions containing aggregated alpha synuclein (αsyn). The progression of parkinsonian pathology and clinical phenotype has been broadly demonstrated to follow a specific pattern, most notably described by Braak and colleagues. In more recent times it has been hypothesized that αsyn itself may be a critical factor in mediating transmission of disease pathology from one brain area to another. Here we investigate the growing body of evidence demonstrating the ability of αsyn to spread transcellularly and induce pathological aggregation affecting neurons by permissive templating and provide a critical analysis of some irregularities in the hypothesis that the progression of PD pathology may be mediated by such a prion-like process. Finally we discuss some key questions that remain unanswered which are vital to determining the potential contribution of a prion-like process to the pathogenesis of PD.
帕金森病（PD）是一种进行性神经退行性疾病，其特征是存在含有聚集的α-突触核蛋白（αsyn）的神经元内包涵体。帕金森病病理和临床表型的进展已被广泛证明遵循特定模式，最著名的是由 Braak 及其同事描述的模式。最近有人假设，αsyn 本身可能是介导疾病病理从一个脑区传播到另一个脑区的关键因素。在这里，我们研究了越来越多的证据，这些证据表明 αsyn 具有通过允许模板形成而进行细胞间传播并诱导影响神经元的病理性聚集的能力，并对该假说的一些不规则性进行了批判性分析，即 PD 病理的进展可能是由这种类朊病毒样过程介导的。最后，我们讨论了一些仍然没有答案的关键问题，这些问题对于确定类朊病毒样过程对 PD 发病机制的潜在贡献至关重要。
