The role of lipid rafts in prion protein biology
The role of lipid rafts in prion protein biology
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DOI：
10.2741/3681
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发表时间：
2011-01-01
影响因子：
3.1
通讯作者：
Hooper, Nigel M.
中科院分区：
文献类型：
--
作者：
Lewis, Victoria;Hooper, Nigel M.
朊蛋白（PrPC）的构象转变为PrPSc是朊病毒疾病的主要致病因素。由于PrPC和PrPSc都与膜相关，因此膜微环境的性质可能在PrPC的构象转换过程以及正常功能中发挥重要作用。在膜内是各种微区，不同的脂质和蛋白质组成的区域，其中最好的研究是富含胆固醇和鞘脂的脂筏。这些结构域的生物化学特征在于它们在低温下对某些洗涤剂的溶解的相对抗性。在这篇文章中，我们审查的证据参与脂筏的本地化和贩运的PrPC，在细胞信号，神经保护和金属结合功能的PrPC，并作为网站的转换PrPC PrPSc和细胞到细胞朊病毒的传输。
The conformational conversion of the cellular prion protein, PrPC, to the misfolded isoform PrPSc is the central pathogenic event in the uniquely transmissible neurodegenerative prion diseases. As both PrPC and PrPSc are associated with membranes, the nature of the membrane microenvironment may well play a significant role in both the conformational conversion process as well as the normal functions of PrPC. Within the membrane are various microdomains, areas of distinct lipid and protein composition, the best studied of which are the cholesterol- and sphingolipid-rich lipid rafts. These domains are characterized biochemically by their relative resistance to solubilization in certain detergents at low temperature. In this article we review the evidence for the involvement of lipid rafts in the localization and trafficking of PrPC, in the cellular signaling, neuroprotective and metal binding functions of PrPC, and as sites for the conversion of PrPC to PrPSc and in cell-to-cell prion transmission.