Ridolfi Elisa, Barone Cinzia, Scarpini Elio, Galimberti Daniela
Neurology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione Cà Granda, IRCCS Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milan, Italy.
Clin Dev Immunol. 2013;2013:939786. doi: 10.1155/2013/939786. Epub 2013 Jul 18.
In the last few years, genetic and biomolecular mechanisms at the basis of Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD) have been unraveled. A key role is played by microglia, which represent the immune effector cells in the central nervous system (CNS). They are extremely sensitive to the environmental changes in the brain and are activated in response to several pathologic events within the CNS, including altered neuronal function, infection, injury, and inflammation. While short-term microglial activity has generally a neuroprotective role, chronic activation has been implicated in the pathogenesis of neurodegenerative disorders, including AD and FTLD. In this framework, the purpose of this review is to give an overview of clinical features, genetics, and novel discoveries on biomolecular pathogenic mechanisms at the basis of these two neurodegenerative diseases and to outline current evidence regarding the role played by activated microglia in their pathogenesis.
在过去几年中,阿尔茨海默病(AD)和额颞叶变性(FTLD)的遗传和生物分子机制已被阐明。小胶质细胞发挥着关键作用,它们是中枢神经系统(CNS)中的免疫效应细胞。它们对大脑中的环境变化极为敏感,并在中枢神经系统内发生的多种病理事件(包括神经元功能改变、感染、损伤和炎症)的刺激下被激活。虽然短期的小胶质细胞活动通常具有神经保护作用,但慢性激活已被认为与神经退行性疾病(包括AD和FTLD)的发病机制有关。在此框架下,本综述的目的是概述这两种神经退行性疾病的临床特征、遗传学以及生物分子致病机制的新发现,并概述关于活化小胶质细胞在其发病机制中所起作用的现有证据。