Prion protein at the crossroads of physiology and disease.

Prion protein at the crossroads of physiology and disease.
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DOI:
10.1016/j.tins.2011.10.002
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发表时间:
2012-02
影响因子:
15.9
通讯作者:
Harris DA
Harris DA
中科院分区:
医学1区
文献类型:
--
作者:
Biasini E;Turnbaugh JA;Unterberger U;Harris DA

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细胞表面存在的细胞朊病毒蛋白(PrPC)是朊病毒神经毒性的关键。虽然一些生物活动已归因于PrPC,其生理功能的明确证明仍然难以捉摸。在这篇综述中,我们将讨论一些建议的功能PrPC,最近建议的作用,在细胞粘附,调节离子电流在细胞膜上,神经保护的重点。我们还将讨论最近的证据支持的想法,PrPC可能作为可溶性低聚物的淀粉样β肽和可能的其他有毒蛋白质聚集体的受体。这些数据表明PrPC的生理功能与其在朊病毒引起的神经退行性疾病中的作用之间存在令人惊讶的新联系。
The presence of the cellular prion protein (PrPC) on the cell surface is critical for the neurotoxicity of prions. Although a number of biological activities have been attributed to PrPC, a definitive demonstration of its physiological function remains elusive. In this review, we will discuss some of the proposed functions of PrPC, focusing on recently suggested roles in cell adhesion, regulation of ionic currents at the cell membrane, and neuroprotection. We will also discuss recent evidence supporting the idea that PrPC may function as a receptor for soluble oligomers of the amyloid β peptide and possibly other toxic protein aggregates. These data suggest surprising new connections between the physiological function of PrPC and its role in neurodegenerative diseases beyond those caused by prions.
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