Guentchev M, Wanschitz J, Voigtländer T, Flicker H, Budka H
Institute of Neurology, University of Vienna, Vienna, Austria.
Am J Pathol. 1999 Nov;155(5):1453-7. doi: 10.1016/S0002-9440(10)65459-4.
Human transmissible spongiform encephalopathies (TSEs) or prion diseases are neurodegenerative disorders of infectious, inherited or sporadic origin and include Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS), kuru and fatal familial insomnia (FFI). Clinicopathologic features of FFI differ markedly from other human TSEs. Previous studies demonstrated selective neuronal vulnerability of parvalbumin positive (PV+) GABAergic inhibitory interneurons in sporadic CJD and experimental TSEs. In this report we show uniform severe loss of PV+ neurons also in other TSEs such as GSS, kuru, new variant and familial CJD. In contrast, these neurons are mostly well preserved, or only moderately reduced, in FFI. Only PV+ neurons surrounded by isolectin-B4 positive perineuronal nets were severely affected in TSEs, suggesting a factor residing in this type of extracellular matrix around PV+ neurons as modulator for the selective neuronal vulnerability.
人类可传播性海绵状脑病（TSEs）或朊病毒病是具有传染性、遗传性或散发性病因的神经退行性疾病，包括克雅氏病（CJD）、格斯特曼-施特劳斯勒-谢inker病（GSS）、库鲁病和致死性家族性失眠症（FFI）。FFI的临床病理特征与其他人类TSEs明显不同。先前的研究表明，在散发性CJD和实验性TSEs中，小白蛋白阳性（PV+）的γ-氨基丁酸能抑制性中间神经元存在选择性神经元易损性。在本报告中，我们表明在其他TSEs如GSS、库鲁病、新型变异型和家族性CJD中，PV+神经元也同样严重缺失。相比之下，在FFI中，这些神经元大多保存完好，或仅轻度减少。在TSEs中，只有被异凝集素-B4阳性神经元周围网包围的PV+神经元受到严重影响，这表明存在于PV+神经元周围这种细胞外基质类型中的一个因素是选择性神经元易损性的调节因子。
