The prion's progress
The prion's progress
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DOI：
10.1038/349569a0
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发表时间：
1991-02
期刊：
影响因子：
64.8
通讯作者：
C. Weissmann
中科院分区：
文献类型：
--
作者：
C. Weissmann
朊病毒从怀疑的斯劳中走出来后，现在正爬上尊重之山。它是否会达到普遍接受的顶峰还有待观察，但最近的几篇文章，包括过去几个月发表的两篇文章，正在加速朊病毒的朝圣之旅。总之，普鲁西纳和他的同事们提出，传播变性性脑病（海绵状脑病）的非常规传染性病原体仅由所谓朊病毒蛋白的一种修饰形式组成，这一观点得到了进一步的实验支持。海绵状脑病包括羊的瘙痒病、人类的克雅氏病和格-斯二氏病，以及疯牛病牛海绵状脑病（bovine spongiform encephalopathy）;所有这些都可以通过实验传播给仓鼠和老鼠。朊病毒蛋白（PrP）是神经元的细胞表面成分，由单个染色体基因编码。然而，来自羊瘙痒病感染脑的PrP部分（称为PrP”）与正常PrP（称为PrP”）的不同之处在于，它不太可溶，并且很大程度上对蛋白酶不敏感。PrP”是PrP的生化前体，但物理性质不同，
THE prion, after emerging from the Slough of Disbelief, is now ascending the Mountain of Respectability. Whether or not it will attain the Pinnacle of Universal Acceptance remains to be seen, but sev-eral recent articles", including two pub-lished in the past couple of months, are speeding the prion on its pilgrimage. In short, the proposal of Prusiner and his colleagues, that the unconventional infectious agent which transmits the degenerative brain diseases known as spongiform encephalopathies consist solely of a modified form of the so-called prion protein, has received further experimental support.Spongiform encephalopathies encompass scrapie in sheep, Creutzfeld–Jakob disease and Gerstmann-Sträussler disease in humans, and BSE (bovine spongiform encephalopathy) in cattle; all of them can be transmitted experimentally to hamsters and mice.“Prion protein'(PrP) is a cell-surface con-stituent of neurons and is encoded by a single chromosomal gene. However, a fraction of PrP from scrapieinfected brain (designated PrP") differs from normal PrP (called PrP") in that it is less soluble and largely resis-tant to proteases. It is PrP" that is proposed to be part or all of the infectious agent; PrP" is the biochemical prccursor to PrP", but the physical difference