Conversion of the BASE prion strain into the BSE strain: The origin of BSE?
Conversion of the BASE prion strain into the BSE strain: The origin of BSE?
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DOI：
10.1371/journal.ppat.0030031
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发表时间：
2007-03-01
期刊：
影响因子：
6.7
通讯作者：
Tagliavini, Fabrizio
中科院分区：
文献类型：
--
作者：
Capobianco, Raffaella;Casalone, Cristina;Tagliavini, Fabrizio
最近在不同国家发现了牛海绵状脑病（BSE）的非典型神经病理学和分子表型。其中一种表型被称为牛“淀粉样”海绵状脑病 (BASE)，它与经典的 BSE 不同，因为它出现了一种独特类型的疾病相关朊病毒蛋白 (PrP)（称为 PrPSc），并且存在 PrP 淀粉样斑块。在这里，我们表明，导致 BSE 和 BASE 的因子在传播到表达 的转基因小鼠后具有不同的生物学特性。 牛 PrP 和非转基因小鼠的近交系。引人注目的是，将 BASE 品系连续传代给非转基因小鼠，诱导出与 BSE 感染小鼠无法区分的神经病理学和分子疾病表型。牛体内存在一种以上与朊病毒病相关的病原体，以及 BASE 菌株转化为 BSE 菌株的能力，可能对 BSE 和海绵状病毒的起源具有重要意义。 其他物种（包括人类）的脑病。
Atypical neuropathological and molecular phenotypes of bovine spongiform encephalopathy (BSE) have recently been identified in different countries. One of these phenotypes, named bovine "amyloidotic'' spongiform encephalopathy (BASE), differs from classical BSE for the occurrence of a distinct type of the disease-associated prion protein (PrP), termed PrPSc, and the presence of PrP amyloid plaques. Here, we show that the agents responsible for BSE and BASE possess different biological properties upon transmission to transgenic mice expressing bovine PrP and inbred lines of nontransgenic mice. Strikingly, serial passages of the BASE strain to nontransgenic mice induced a neuropathological and molecular disease phenotype indistinguishable from that of BSE- infected mice. The existence of more than one agent associated with prion disease in cattle and the ability of the BASE strain to convert into the BSE strain may have important implications with respect to the origin of BSE and spongiform encephalopathies in other species, including humans.