Center for Alzheimer's and Neurodegenerative Diseases, Peter O'Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center, NL10.120, 6000 Harry Hines Blvd., Dallas, TX, 75390, USA.
Alzheimer's Disease and Chronic Traumatic Encephalopathy Center, Boston University School of Medicine, Boston University, Boston, MA, USA.
Acta Neuropathol. 2021 Dec;142(6):951-960. doi: 10.1007/s00401-021-02373-5. Epub 2021 Oct 9.
Chronic traumatic encephalopathy (CTE), a neurodegenerative tauopathy, is associated with behavioral, mood and cognitive impairment, including dementia. Tauopathies are neurodegenerative diseases whose neuropathological phenotypes are characterized by distinct histopathologic features of tau pathology, which progressively deposit throughout the brain. In certain tauopathies, especially Alzheimer's disease (AD), tau deposition appears to follow brain network connections. Experimental evidence suggests that the progression of tau pathology in humans, mouse and cell models could be explained by tau seeds that adopt distinct conformations and serve as templates for their own amplification to mediate transcellular propagation of pathology. Tau seeds are efficiently detected by the induction of aggregation in cell-based "biosensors" that express tau repeat domain (RD) with a disease-associated mutation (P301S) fused to complementary fluorescent protein tags (cyan and yellow fluorescent protein). Biosensors enable quantification of tau seeding in fixed and fresh-frozen brain tissue. Phospho-tau deposition in CTE follows progressive stages (I-IV), but the relationship of seeding to this deposition is unclear. We have used an established biosensor assay to independently quantify tau seeding as compared to AT8 phospho-tau histopathology in thin sections of fixed tissues of 11 brain regions from 27 patients with CTE, 5 with other tauopathies, and 5 negative controls. In contrast to prior studies of AD, we detected tau seeding late in the course of CTE (predominantly stages III and IV). It was less anatomically prevalent than AT8-positive inclusions, which were relatively widespread. We especially observed seeding in the limbic system (amygdala, thalamus, basal ganglia), which may explain the dominant cognitive and behavior impairments that characterize CTE.
慢性创伤性脑病(CTE)是一种神经退行性tau 病,与行为、情绪和认知障碍有关,包括痴呆。tau 病是神经退行性疾病,其神经病理学表型的特征是 tau 病理学的独特组织病理学特征,这些特征逐渐在整个大脑中沉积。在某些 tau 病中,特别是阿尔茨海默病(AD)中,tau 沉积似乎遵循大脑网络连接。实验证据表明,人类、小鼠和细胞模型中 tau 病理学的进展可以用 tau 种子来解释,这些种子采用不同的构象,并作为自身扩增的模板,介导病理学的细胞间传播。tau 种子可以通过在表达与疾病相关突变(P301S)融合互补荧光蛋白标签(青色和黄色荧光蛋白)的 tau 重复结构域(RD)的细胞“生物传感器”中诱导聚集来有效地检测到。生物传感器可用于定量固定和新鲜冷冻脑组织中的 tau 种子。CTE 中的磷酸化 tau 沉积遵循渐进阶段(I-IV),但种子与这种沉积的关系尚不清楚。我们使用已建立的生物传感器测定法,独立地定量比较了在 27 例 CTE 患者、5 例其他 tau 病患者和 5 例阴性对照患者的 11 个脑区固定组织的薄切片中的 tau 种子与 AT8 磷酸化 tau 组织病理学,这些患者的组织切片均经过固定和新鲜冷冻处理。与 AD 的先前研究相比,我们在 CTE 的过程中晚期检测到 tau 种子(主要是阶段 III 和 IV)。与 AT8 阳性包涵体相比,它在解剖上的分布较少,后者相对广泛。我们特别观察到在边缘系统(杏仁核、丘脑、基底神经节)中存在种子,这可能解释了 CTE 特征性的认知和行为障碍。