Prions are novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease.
Prions are novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease.
复制标题
朊病毒是引起痒病和克雅氏病的新型传染性病原体。
DOI：
10.1002/bies.950050612
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Prusiner,SB
中科院分区：
文献类型：
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作者：
Prusiner,SB
瘙痒病和克雅氏病(CJD)是由蛋白引起的，似乎既不同于病毒，也不同于类病毒。Prion含有传染性所需的蛋白质，但尚未在其中发现核酸。Prion蛋白是由细胞基因编码的，而不是由具有传染性的Prion颗粒内的核酸编码的。已经确定了普里恩蛋白的细胞同源物。这种同系物在新陈代谢中的作用尚不清楚。Pron蛋白，而不是细胞同系物，聚集成杆状颗粒，在组织化学和超微结构上与淀粉样蛋白相同。在瘙痒病和CJD感染的啮齿动物大脑以及CJD感染的人脑中，Pron蛋白的细胞外集合形成淀粉样斑块。在斑块内，蛋白聚集形成淀粉样细丝。阐明普恩蛋白与其细胞同源蛋白之间的分子差异对于理解普恩的化学结构和复制具有重要意义。
Scrapie and Creutzfeldt–Jakob disease (CJD) are caused by prions, which appear to be different from both viruses and viroids. Prions contain protein which is required for infectivity, but no nucleic acid has been found within them. Prion proteins are encoded by a cellular gene and not by a nucleic acid within the infectious prion particle. A cellular homologue of the prion protein has been IDentified. The role of this homologue in metabolism is unknown. Prion proteins, but not the cellular homologue, aggregate into rod‐shaped particles that are histo‐chemically and ultrastructurally IDentical to amyloid. Extracellular collections of prion proteins form amyloid plaques in scrapie‐ and CJD‐infected rodent brains as well as CJD‐infected human brains. Within the plaques, prion proteins assemble to form amyloid filaments. Elucidating the molecular differences between the prion protein and its cellular homologue may be important in understanding the chemical structure and replication of prions.