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Using NMR spectroscopy to investigate the role played by copper in prion diseases.
Neurological Sciences ( IF 3.3 ) Pub Date : 2020-04-24 , DOI: 10.1007/s10072-020-04321-9
Rawiah A Alsiary 1 , Mawadda Alghrably 2 , Abdelhamid Saoudi 3 , Suliman Al-Ghamdi 3 , Lukasz Jaremko 2 , Mariusz Jaremko 2 , Abdul-Hamid Emwas 4
Affiliation  

Prion diseases are a group of rare neurodegenerative disorders that develop as a result of the conformational conversion of normal prion protein (PrPC) to the disease-associated isoform (PrPSc). The mechanism that actually causes disease remains unclear. However, the mechanism underlying the conformational transformation of prion protein is partially understood-in particular, there is strong evidence that copper ions play a significant functional role in prion proteins and in their conformational conversion. Various models of the interaction of copper ions with prion proteins have been proposed for the Cu (II)-binding, cell-surface glycoprotein known as prion protein (PrP). Changes in the concentration of copper ions in the brain have been associated with prion diseases and there is strong evidence that copper plays a significant functional role in the conformational conversion of PrP. Nevertheless, because copper ions have been shown to have both a positive and negative effect on prion disease onset, the role played by Cu (II) ions in these diseases remains a topic of debate. Because of the unique properties of paramagnetic Cu (II) ions in the magnetic field, their interactions with PrP can be tracked even at single atom resolution using nuclear magnetic resonance (NMR) spectroscopy. Various NMR approaches have been utilized to study the kinetic, thermodynamic, and structural properties of Cu (II)-PrP interactions. Here, we highlight the different models of copper interactions with PrP with particular focus on studies that use NMR spectroscopy to investigate the role played by copper ions in prion diseases.

中文翻译:

使用NMR光谱研究铜在病毒疾病中的作用。

on病毒是一组罕见的神经退行性疾病,是由于正常病毒蛋白(PrPC)构象转化为与疾病相关的同工型(PrPSc)所致。实际导致疾病的机制仍不清楚。然而,部分理解了the病毒蛋白构象转化的机制,尤其是有强有力的证据表明,铜离子在病毒蛋白及其构象转化中起着重要的作用。已经提出了铜离子与病毒蛋白相互作用的各种模型,用于铜(II)结合的细胞表面糖蛋白,称为病毒蛋白(PrP)。脑中铜离子浓度的变化与病毒疾病有关,并且有充分的证据表明铜在PrP的构象转化中起着重要的作用。但是,由于铜离子已显示出对病毒疾病发作的正面和负面影响,因此铜离子在这些疾病中的作用仍然是一个争论的话题。由于顺磁性Cu(II)离子在磁场中的独特性质,甚至可以使用核磁共振(NMR)光谱以单原子分辨率跟踪它们与PrP的相互作用。各种NMR方法已用于研究Cu(II)-PrP相互作用的动力学,热力学和结构性质。这里,
更新日期:2020-04-24
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