Department of Neuroscience, Genentech Inc., 1 DNA Way, South San Francisco, CA 94080, USA.
Department of Biomedical Imaging, Genentech Inc., 1 DNA Way, South San Francisco, CA 94080, USA.
Cell Rep. 2019 Aug 20;28(8):2111-2123.e6. doi: 10.1016/j.celrep.2019.07.060.
Complement pathway overactivation can lead to neuronal damage in various neurological diseases. Although Alzheimer's disease (AD) is characterized by β-amyloid plaques and tau tangles, previous work examining complement has largely focused on amyloidosis models. We find that glial cells show increased expression of classical complement components and the central component C3 in mouse models of amyloidosis (PS2APP) and more extensively tauopathy (TauP301S). Blocking complement function by deleting C3 rescues plaque-associated synapse loss in PS2APP mice and ameliorates neuron loss and brain atrophy in TauP301S mice, improving neurophysiological and behavioral measurements. In addition, C3 protein is elevated in AD patient brains, including at synapses, and levels and processing of C3 are increased in AD patient CSF and correlate with tau. These results demonstrate that complement activation contributes to neurodegeneration caused by tau pathology and suggest that blocking C3 function might be protective in AD and other tauopathies.
补体途径过度激活可导致各种神经退行性疾病中的神经元损伤。虽然阿尔茨海默病(AD)的特征是β-淀粉样斑块和tau 缠结,但之前对补体的研究主要集中在淀粉样变性模型上。我们发现,神经胶质细胞在淀粉样变性模型(PS2APP)和更广泛的 tau 病(TauP301S)小鼠模型中显示出经典补体成分和中央成分 C3 的表达增加。通过删除 C3 来阻断补体功能可挽救 PS2APP 小鼠中斑块相关的突触丢失,并改善 TauP301S 小鼠中的神经元丢失和脑萎缩,改善神经生理和行为测量。此外,C3 蛋白在 AD 患者的大脑中升高,包括在突触中,并且 C3 的水平和处理在 AD 患者的 CSF 中增加,并与 tau 相关。这些结果表明补体激活导致 tau 病理学引起的神经退行性变,并表明阻断 C3 功能可能对 AD 和其他 tau 病具有保护作用。