Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland.
Neuron. 2009 Dec 24;64(6):783-90. doi: 10.1016/j.neuron.2009.12.016.
Recent reports indicate that a growing number of intracellular proteins are not only prone to pathological aggregation but can also be released and "infect" neighboring cells. Therefore, many complex diseases may obey a simple model of propagation where the penetration of seeds into hosts determines spatial spread and disease progression. We term these proteins prionoids, as they appear to infect their neighbors just like prions--but how can bulky protein aggregates be released from cells and how do they access other cells? The widespread existence of such prionoids raises unexpected issues that question our understanding of basic cell biology.
最近的报告表明，越来越多的细胞内蛋白质不仅容易发生病理性聚集，而且还可以被释放出来并“感染”邻近的细胞。因此，许多复杂的疾病可能遵循一个简单的传播模式，即种子进入宿主决定了空间传播和疾病进展。我们将这些蛋白质称为朊病毒样蛋白，因为它们似乎像朊病毒一样感染它们的邻居——但是，大的蛋白质聚集体如何从细胞中释放出来，它们又如何进入其他细胞？这些朊病毒样蛋白的广泛存在引发了一些意想不到的问题，这些问题对我们对基础细胞生物学的理解提出了质疑。
