朊病毒蛋白prion与二价铜离子相互作用的研究进展

Research Progress in the Interaction of Prion Proteins and Copper(Ⅱ) Ions

传染性海绵状脑病是一类致死性的中枢神经系统退行性疾病,其发病机制与prion蛋白构象的错误折叠相关。Prion蛋白对二价铜离子具有极强的选择性。文章综述了二价铜离子与PrPC的结合模式、结合力以及铜离子在prion蛋白错误折叠过程中可能发挥的作用。

Prion diseases or transmissible spongiform encephalopathies are a group of rare and fatal neurodegenerative disorders that can be sporadic,inherited, or acquired by infectious means. These include Creutzfeldt-Jacob disease, kuru, familial fatal insomnia in humans, scrapie in sheep and goats, and bovine spongiform encephalopathies in cattle. The diseases are associated with a conformational conversion of the normal cellular, membrane-anchored prion protein （PrPc） into an oligomerie, [3-sheet rich, proteinase-K resistant,infectious form termed PRPso. An enormous body of literature has been published in the last ten years concerning copper and prion protein. This present review attempts to look at the evidence for co-ordination, affinity and the physiology function of copper binding to PrP. The evidence with possible roles for PrP when bound to copper was connected here. No clear conclusions can be made from the available data, hut it is clear from the present review that the aspects of copper association with PrP need to he re-investigated.