Department of Microbiology and Microbial Biotechnology, Faculty of Life Science and Biotechnology, Shahid Beheshti University, Tehran 1983969411, Iran.
Department of Experimental and Clinical Biomedical Sciences, University of Florence, Viale G.B Morgagni 50, 50134 Florence, Italy.
Int J Mol Sci. 2020 Oct 1;21(19):7273. doi: 10.3390/ijms21197273.
Alzheimer's disease (AD) is the most prevalent form of dementia and soluble amyloid β (Aβ) oligomers are thought to play a critical role in AD pathogenesis. Cellular prion protein (PrP) is a high-affinity receptor for Aβ oligomers and mediates some of their toxic effects. The -terminal region of PrP can interact with Aβ, particularly the region encompassing residues 95-110. In this study, we identified a soluble and unstructured prion-derived peptide (PrP) that is external to this region of the sequence and was found to successfully reduce the mitochondrial impairment, intracellular ROS generation and cytosolic Ca uptake induced by oligomeric Aβ ADDLs in neuroblastoma SH-SY5Y cells. PrP was also found to rescue SH-SY5Y cells from Aβ ADDL internalization. The peptide did not change the structure and aggregation pathway of Aβ ADDLs, did not show co-localization with Aβ ADDLs in the cells and showed a partial colocalization with the endogenous cellular PrP. As a sequence region that is not involved in Aβ binding but in PrP self-recognition, the peptide was suggested to protect against the toxicity of Aβ oligomers by interfering with cellular PrP and/or activating a signaling that protected the cells. These results strongly suggest that PrP has therapeutic potential for AD.
阿尔茨海默病(AD)是最常见的痴呆症形式,可溶性淀粉样β(Aβ)寡聚体被认为在 AD 发病机制中起关键作用。细胞朊蛋白(PrP)是 Aβ寡聚体的高亲和力受体,介导其一些毒性作用。PrP 的 N 端区域可以与 Aβ相互作用,特别是包含残基 95-110 的区域。在这项研究中,我们鉴定了一种可溶性的、无结构的朊病毒衍生肽(PrP),它位于该序列区域之外,并且成功地减少了神经母细胞瘤 SH-SY5Y 细胞中由寡聚 Aβ ADDLs 引起的线粒体损伤、细胞内 ROS 生成和胞质 Ca 摄取。PrP 还能挽救 SH-SY5Y 细胞免受 Aβ ADDL 的内化。该肽不改变 Aβ ADDLs 的结构和聚集途径,不在细胞内与 Aβ ADDLs 共定位,与内源性细胞 PrP 部分共定位。由于该肽不参与 Aβ 结合,而是参与 PrP 自我识别的序列区域,因此推测该肽通过干扰细胞 PrP 和/或激活保护细胞的信号通路来防止 Aβ 寡聚体的毒性。这些结果强烈表明 PrP 对 AD 具有治疗潜力。