Bergström A L, Heegaard P M H, Dyrbye H, Lind P, Laursen H
National Veterinary Institute, The Technical University of Denmark.
Clin Neuropathol. 2009 Sep-Oct;28(5):321-32.
The transmissible spongiform encephalopathies are characterized by vacuolization, neuronal loss, gliosis and deposition of a misfolded and Proteinase K resistant isoform of the prion protein (PrP(Sc)) in the central nervous system. METHODS MATERIALS AND PATIENTS: Paraffin-embedded tissue blot (PET-blot), immunohistochemistry (IHC) and Western blotting (WB) were combined to study the morphology and localization of disease related PrP in Danish patients with different subtypes of sporadic Creutzfeldt-Jakob disease, familiar Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease.
There was a good morphological and anatomical concordance between what was found with PET-blot and IHC in all patients. In some specific cases, the PET-blot was superior to IHC in sensitivity. To our knowledge, this is the first report where PET-blot analysis is applied to hereditary forms of human transmissible spongiform encephalopathies and compared with sporadic cases of Creutzfeldt-Jakob disease.
传染性海绵状脑病的特征为中枢神经系统出现空泡化、神经元丢失、胶质细胞增生以及一种错误折叠且对蛋白酶K具有抗性的朊蛋白异构体（PrP(Sc)）沉积。方法、材料与患者：结合石蜡包埋组织印迹（PET印迹）、免疫组织化学（IHC）和蛋白质印迹法（WB），研究丹麦散发性克雅氏病不同亚型、家族性克雅氏病和格斯特曼-施特劳斯勒-谢因克综合征患者中与疾病相关的PrP的形态和定位。
在所有患者中，PET印迹和IHC所发现的结果在形态和解剖学上具有良好的一致性。在某些特定病例中，PET印迹在敏感性方面优于IHC。据我们所知，这是首次将PET印迹分析应用于人类遗传性传染性海绵状脑病并与散发性克雅氏病病例进行比较的报告。
