骨和脊髓神经的明显参与进步的超核性麻
Hidetomo Tanaka1, Ivan Martinez-Valbuena1, Shelley L Forrest1,2
1Department of Laboratory Medicine and Pathobiology and Tanz Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto, Ontario M5T 0S8, Canada.
周围神经系统的 (PNS-tau) 在渐进性上核性麻 (PSP) 中突出,使其与其他病区别开来. 这一发现表明PNS-tau可能对PSP诊断具有临床和生物标志物的相关性.
科学领域:
- 神经科学是一个神经科学.
- 神经病理学神经病理学
背景情况:
- 神经退行性蛋白质病变通常涉及错误折叠的或α-synuclein聚合物.
- 周围神经系统 (PNS) 聚合物在α-synucleinopathies中已知,但在tauopathies中研究较少.
研究的目的:
- 为了研究和表征外周神经系统 (PNS-tau) 中的病理,跨越各种病症.
- 确定PNS-tau是否可以作为不同病症的区分生物标志物.
主要方法:
- 通过免疫组织化学,Gallyas银色染色,生物化学和播种分析,对54例陶氏病变病例的头骨和脊髓神经进行了陶氏病理学的检查.
- 包括渐进性超核性 (PSP),阿尔茨海默病 (AD),皮质核退行 (CBD),皮克病等病例.
主要成果:
- 在大多数PSP病例 (88-100%) 的PNS中发现了显著的酸化和4重复矿.
- 在CBD中,PNS中的tau病理很少见,在AD,CTE或Pick病中没有明显.
- 第一次使用tau生物传感器细胞在PNS中证明了tau的播种能力.
结论:
- 显著的PNS-tau病理是PSP与其他病症相比的一个显著特征.
- 在PSP和CBD之间PNS-tau的形态差异可能反映了中枢神经系统的病理.
- 在PSP中PNS-tau的高频率和早期存在表明了潜在的临床和生物标志物的实用性.
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