Clinical Neuroscience Unit, Department of Neurology, University of Bonn, Sigmund-Freud-Strasse 25, 53127 Bonn, Germany.
Nature. 2013 Jan 31;493(7434):674-8. doi: 10.1038/nature11729. Epub 2012 Dec 19.
Alzheimer's disease is the world's most common dementing illness. Deposition of amyloid-β peptide drives cerebral neuroinflammation by activating microglia. Indeed, amyloid-β activation of the NLRP3 inflammasome in microglia is fundamental for interleukin-1β maturation and subsequent inflammatory events. However, it remains unknown whether NLRP3 activation contributes to Alzheimer's disease in vivo. Here we demonstrate strongly enhanced active caspase-1 expression in human mild cognitive impairment and brains with Alzheimer's disease, suggesting a role for the inflammasome in this neurodegenerative disease. Nlrp3(-/-) or Casp1(-/-) mice carrying mutations associated with familial Alzheimer's disease were largely protected from loss of spatial memory and other sequelae associated with Alzheimer's disease, and demonstrated reduced brain caspase-1 and interleukin-1β activation as well as enhanced amyloid-β clearance. Furthermore, NLRP3 inflammasome deficiency skewed microglial cells to an M2 phenotype and resulted in the decreased deposition of amyloid-β in the APP/PS1 model of Alzheimer's disease. These results show an important role for the NLRP3/caspase-1 axis in the pathogenesis of Alzheimer's disease, and suggest that NLRP3 inflammasome inhibition represents a new therapeutic intervention for the disease.
阿尔茨海默病是世界上最常见的痴呆症。淀粉样蛋白-β肽的沉积通过激活小胶质细胞驱动大脑神经炎症。事实上,淀粉样蛋白-β激活小胶质细胞中的 NLRP3 炎性小体对于白细胞介素-1β的成熟和随后的炎症事件是至关重要的。然而,NLRP3 的激活是否导致体内阿尔茨海默病仍不清楚。在这里,我们在人类轻度认知障碍和阿尔茨海默病患者的大脑中强烈地增强了活性半胱天冬酶-1的表达,这表明炎性小体在这种神经退行性疾病中具有作用。携带与家族性阿尔茨海默病相关突变的 Nlrp3(-/-)或 Casp1(-/-) 小鼠在很大程度上免受空间记忆丧失和其他与阿尔茨海默病相关的后遗症的影响,并且显示出大脑半胱天冬酶-1和白细胞介素-1β的激活减少以及淀粉样蛋白-β清除增加。此外,NLRP3 炎性小体缺陷使小胶质细胞向 M2 表型倾斜,并导致 APP/PS1 阿尔茨海默病模型中淀粉样蛋白-β的沉积减少。这些结果表明 NLRP3/caspase-1 轴在阿尔茨海默病的发病机制中具有重要作用,并表明 NLRP3 炎性小体抑制代表了该疾病的一种新的治疗干预措施。