牛朊病毒疾病中的不同分子表型。
Distinct molecular phenotypes in bovine prion diseases.
作者信息
Biacabe Anne-Gaëlle, Laplanche Jean-Louis, Ryder Stephen, Baron Thierry
机构信息
AFSSA-Lyon, Unité 'Virologie-ATNC', 31 Avenue Tony Garnier, 69364 Lyon Cedex 07, France.
出版信息
EMBO Rep. 2004 Jan;5(1):110-5. doi: 10.1038/sj.embor.7400054.
Abstract
Bovine spongiform encephalopathy (BSE) in cattle, the most likely cause of variant Creutzfeldt-Jakob disease in humans, is thought to be caused by a unique infectious agent, with stable features, even when transmitted to other species. Here, we show the existence of an atypical molecular phenotype among cattle diagnosed with BSE in France. Following western blot analysis, three cases showed unusual features of the electrophoretic profiles of the protease-resistant prion protein (PrP(res)) accumulating in the brain. The PrP(res) patterns were similar in these three atypical cases, showing a higher molecular mass of unglycosylated PrP(res) and strong labelling by P4 monoclonal antibody compared to 55 typical BSE cases. This finding suggests either some phenotypic modifications of PrP(res) following infection by the BSE agent or the existence of alternative origins of such diseases in cattle.
摘要
牛海绵状脑病（BSE）是牛的一种疾病，被认为是人类变异型克雅氏病最可能的病因，它被认为是由一种独特的传染性病原体引起的，即使传播到其他物种，也具有稳定的特征。在这里，我们展示了在法国被诊断患有BSE的牛中存在一种非典型分子表型。经过蛋白质印迹分析，三例显示出在脑中积累的抗蛋白酶朊病毒蛋白（PrP(res)）的电泳图谱具有异常特征。这三例非典型病例中的PrP(res)模式相似，与55例典型BSE病例相比，未糖基化的PrP(res)分子量更高，且被P4单克隆抗体强烈标记。这一发现表明，要么是BSE病原体感染后PrP(res)发生了一些表型改变，要么是牛中存在此类疾病的其他起源。
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