Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease.
Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease.
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变异型、医源性和散发型克雅氏病患者骨骼肌中 PrPSc 的检测和定位。
DOI：
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发表时间：
2006
影响因子：
6
通讯作者：
J. Ironside
中科院分区：
文献类型：
--
作者：
A. Peden;D. Ritchie;M. Head;J. Ironside
变异型克雅氏病（vCJD）与其他人类朊病毒疾病的不同之处在于，致病性朊病毒蛋白PrP（Sc）可以在整个身体的神经元部位，主要是淋巴组织内检测到更大程度。然而，最近一项使用高灵敏度蛋白质印迹技术的研究显示，四分之一的瑞士散发性CJD（sCJD）患者骨骼肌中PrP（Sc）水平较低。这就提出了一个问题，即肌肉中的PrP（Sc）是否也可以在其他人群的vCJD、sCJD和医源性（iCJD）患者中检测到。因此，我们已经使用了相同的高灵敏度的蛋白质印迹技术，结合石蜡包埋的组织印迹，筛选PrP（Sc）在肌肉组织标本，从49克雅氏病患者在英国尸检。这些技术确定了肌肉PrP（Sc）在8的17 vCJD，7的26 sCJD，和2的5 iCJD患者。石蜡包埋组织印迹分析显示PrP（Sc）在骨骼肌局部解剖结构中具有神经纤维的形态学和免疫组织化学特征。从所有形式的克雅氏病的肌肉组织中的PrP（Sc）的检测表明，在这些组织中可能存在的感染性，这表明重要的影响，评估通过污染的手术器械医源性传播的潜在风险。
Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that the pathogenic prion protein PrP(Sc) can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. However, a recent study using a high-sensitivity Western blotting technique revealed low levels of PrP(Sc) in skeletal muscle from a quarter of Swiss patients with sporadic CJD (sCJD). This posed the question of whether PrP(Sc) in muscle could also be detected in vCJD, sCJD, and iatrogenic (iCJD) patients from other populations. Therefore, we have used the same high-sensitivity Western blotting technique, in combination with paraffin-embedded tissue blotting, to screen for PrP(Sc) in muscle tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom. These techniques identified muscle PrP(Sc) in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients. Paraffin-embedded tissue blotting analysis showed PrP(Sc) in skeletal muscle in localized anatomical structures that had the morphological and immunohistochemical characteristics of nerve fibers. The detection of PrP(Sc) in muscle tissue from all forms of CJD indicates the possible presence of infectivity in these tissues, suggesting important implications for assessing the potential risk of iatrogenic spread via contaminated surgical instruments.
DOI：
10.1073/pnas.97.18.10168
发表时间：
2000-08-29
影响因子：
11.1
作者：
Parchi, P;Zou, WQ;Chen, SG
通讯作者：
Chen, SG