Prion diseases, also known as Transmissible Spongiform Encephalopathies (TSEs), are protein-based neurodegenerative disorders (NDs) affecting humans and animals. They are characterized by the conformational conversion of the normal cellular prion protein, PrP, into the pathogenic isoform, PrP. Prion diseases are invariably fatal and despite ongoing research, no effective prophylactic or therapeutic avenues are currently available. Anthocyanins (ACNs) are unique flavonoid compounds and interest in their use as potential neuroprotective and/or therapeutic agents against NDs, has increased significantly in recent years. Therefore, we investigated the potential anti-oxidant and anti-prion effects of Oenin and Myrtillin, two of the most common anthocyanins, using the most accepted in the field overexpressing PrP model and a cell free protein aggregation model. Our results, indicate both anthocyanins as strong anti-oxidant compounds, upregulating the expression of genes involved in the anti-oxidant response, and reducing the levels of Reactive Oxygen Species (ROS), produced due to pathogenic prion infection, through the activation of the pathway. Importantly, they showcased remarkable anti-prion potential, as they not only caused the clearance of pathogenic PrP aggregates, but also completely inhibited the formation of PrP fibrils in the Cerebrospinal Fluid (CSF) of patients with Creutzfeldt–Jakob disease (CJD). Therefore, Oenin and Myrtillin possess pleiotropic effects, suggesting their potential use as promising preventive and/or therapeutic agents in prion diseases and possibly in the spectrum of neurodegenerative proteinopathies.

中文翻译：

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花青素的抗朊病毒作用


朊病毒病，也称为传染性海绵状脑病 (TSE)，是一种影响人类和动物的基于蛋白质的神经退行性疾病 (ND)。它们的特征是正常细胞朊病毒蛋白 PrP 构象转变为致病亚型 PrP。朊病毒疾病总是致命的，尽管正在进行研究，但目前还没有有效的预防或治疗途径。花青素 (ACN) 是一种独特的黄酮类化合物，近年来人们对其作为潜在的神经保护剂和/或 ND 治疗剂的兴趣显着增加。因此，我们使用该领域最受认可的过表达 PrP 模型和无细胞蛋白聚集模型，研究了 Oenin 和 Myrtillin（两种最常见的花青素）的潜在抗氧化和抗朊病毒作用。我们的研究结果表明，花青素作为强抗氧化化合物，通过激活途径。重要的是，它们表现出显着的抗朊病毒潜力，因为它们不仅能清除致病性 PrP 聚集物，还能完全抑制克雅氏病 (CJD) 患者脑脊液 (CSF) 中 PrP 原纤维的形成。因此，Oenin 和 Myrtillin 具有多效性，表明它们有可能用作朊病毒疾病以及可能在神经退行性蛋白质病中的有前途的预防和/或治疗剂。