Département de Psychiatrie & Neurosciences, Faculté de Médecine, Université Laval, Québec, QC, G1V 0A6, Canada.
Centre de Recherche du CHU de Québec, Axe Neuroscience, T2-50, 2705 boulevard Laurier, Québec, QC, G1V 4G2, Canada.
J Neurol. 2021 Sep;268(9):3493-3504. doi: 10.1007/s00415-021-10418-8. Epub 2021 Feb 24.
Decades of research on the prion protein and its associated diseases have caused a paradigm shift in our understanding of infectious agents. More recent years have been marked by a surge of studies supporting the application of these findings to a broad array of neurodegenerative disorders such as Alzheimer's and Parkinson's diseases. Here, we present evidence to suggest that Huntington's disease, a monogenic disorder of the central nervous system, shares features with prion disorders and that, it too, may be governed by similar mechanisms. We further posit that these similarities could suggest that, like other common neurodegenerative disorders, sporadic forms of Huntington's disease may exist.
几十年来，对朊病毒蛋白及其相关疾病的研究导致了我们对传染性病原体的理解发生了范式转变。近年来，大量研究支持将这些发现应用于广泛的神经退行性疾病，如阿尔茨海默病和帕金森病。在这里，我们提出证据表明，亨廷顿病是一种中枢神经系统的单基因疾病，与朊病毒疾病具有相似的特征，并且也可能受到类似机制的控制。我们进一步假设，这些相似性可能表明，像其他常见的神经退行性疾病一样，散发性亨廷顿病可能存在。
