Sikorska Beata, Gajos Agata, Bogucki Andrzej, Zielonka Emil, Sigurdson Christina, Liberski Pawel P
a Department of Molecular Pathology and Neuropathology , Medical University of Lodz , Kosciuszki 4 st, Lodz , Poland.
b Department of Extrapyramidal Diseases , Medical University of Lodz , Kosciuszki 4 st, Lodz , Poland.
Prion. 2017 Nov 2;11(6):431-439. doi: 10.1080/19336896.2017.1384109.
We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmitted to Tg20 transgenic mice overexpressing prion protein (PrP). We identified three main types of amyloid deposits in mCWD: large amyloid deposits, unicentric plaques similar to kuru plaques in human prion diseases and multicentric plaques reminiscent of plaques typical of GSS. The most unique type of plaques were large subpial amyloid deposits. They were composed of large areas of amyloid fibrils but did not form "star-like" appearances of unicentric plaques. All types of plaques were totally devoid of dystrophic neuritic elements. However, numerous microglial cells invaded them. The plaques observed by confocal laser microscope were of the same types as those analyzed by electron microscopy. Neuronal processes surrounding the plaques did not show typical features of neuroaxonal dystrophy.
我们在此报告慢性消耗病（CWD）传播至过度表达朊病毒蛋白（PrP）的Tg20转基因小鼠后，其淀粉样斑块的超微结构。我们在小鼠慢性消耗病（mCWD）中鉴定出三种主要类型的淀粉样沉积物：大型淀粉样沉积物、类似于人类朊病毒病中库鲁病斑块的单中心斑块，以及让人联想到格斯特曼综合征（GSS）典型斑块的多中心斑块。最独特的斑块类型是大型软膜下淀粉样沉积物。它们由大片淀粉样纤维组成，但未形成单中心斑块的“星状”外观。所有类型的斑块均完全没有营养不良性神经突成分。然而，大量小胶质细胞侵入其中。共聚焦激光显微镜观察到的斑块与电子显微镜分析的斑块类型相同。斑块周围的神经元突起未显示出神经轴突营养不良的典型特征。
