Rogers Joseph, Mastroeni Diego, Leonard Brian, Joyce Jeffrey, Grover Andrew
The L. J. Roberts Center for Alzheimer's Research, Sun Health Research Institute Sun City, Arizona 85351, USA.
Int Rev Neurobiol. 2007;82:235-46. doi: 10.1016/S0074-7742(07)82012-5.
Microglial activation similar to that which occurs in peripheral macrophages during inflammatory attack was first demonstrated in the Alzheimer's disease (AD) brain two decades ago. Localization to pathologically vulnerable regions of AD cortex, localization to sites of specific AD pathology such as amyloid-beta peptide (Abeta) deposits, and the ability of activated microglia to release toxic inflammatory factors suggested that the activation of microglia in AD might play a pathogenic role. However, proving this hypothesis in a disease in which so many profound pathologies occur (e.g., Abeta deposition, neurofibrillary tangle formation, inflammation, neuronal loss, neuritic loss, synaptic loss, neuronal dysfunction, vascular alterations) has proven difficult. Although investigations of microglia in Parkinson's disease (PD) are more recent and therefore less extensive, demonstration of a pathogenic role for microglial activation may actually be much simpler in PD than AD because the root pathological event in PD, loss of dopamine (DA)-secreting substantia nigra neurons, is already well established. Indeed, indirect but converging evidence of a pathogenic role for activated microglia in PD has already begun to emerge. The nigra reportedly has the highest density of microglia in brain, and, in PD, nigral microglia are not only highly activated but also highly clustered around dystrophic DA neurons. 6-OHDA and MPTP models of PD in rodents induce substantia nigra microglial activation. More cogent, injections of the classic microglial/macrophage activator lipopolysaccharide into or near the rodent nigra cause a specific loss of DA neurons there. Culture models with human microglia and human cellular targets replicate this phenomenon. Notably, nearly all the proposed etiologies of PD, including brain bacterial and viral exposure, pesticides, drug contaminants, and repeated head trauma, are known to cause brain inflammation. A mechanism by which activated microglia might specifically target DA neurons remains a critical missing link in the proof of a pathogenic role for activated microglia in PD. If such a link could be established, however, clinical intervention trials with agents that dampen microglial activation might be warranted in PD.
二十年前,在阿尔茨海默病(AD)大脑中首次证实了与炎症攻击期间外周巨噬细胞中发生的情况类似的小胶质细胞激活。小胶质细胞定位于AD皮质的病理易损区域,定位于特定AD病理部位,如β-淀粉样肽(Aβ)沉积部位,以及活化的小胶质细胞释放有毒炎症因子的能力,表明AD中小胶质细胞的激活可能起致病作用。然而,在一种出现如此多严重病理变化(如Aβ沉积、神经原纤维缠结形成、炎症、神经元丧失、神经突丧失、突触丧失、神经元功能障碍、血管改变)的疾病中证明这一假设已被证明是困难的。尽管对帕金森病(PD)中小胶质细胞的研究相对较新,因此范围也较小,但在PD中证明小胶质细胞激活的致病作用实际上可能比AD简单得多,因为PD的根本病理事件,即分泌多巴胺(DA)的黑质神经元丧失,已经得到充分证实。事实上,关于活化的小胶质细胞在PD中起致病作用的间接但相互印证的证据已经开始出现。据报道,黑质是大脑中小胶质细胞密度最高的区域,在PD中,黑质小胶质细胞不仅高度活化,而且高度聚集在营养不良的DA神经元周围。啮齿动物的PD的6-羟基多巴胺(6-OHDA)和1-甲基-4-苯基-1,2,3,6-四氢吡啶(MPTP)模型可诱导黑质小胶质细胞激活。更有说服力的是,将经典的小胶质细胞/巨噬细胞激活剂脂多糖注射到啮齿动物黑质内或其附近会导致那里的DA神经元特异性丧失。用人小胶质细胞和人细胞靶点的培养模型复制了这一现象。值得注意的是,几乎所有提出的PD病因,包括脑部细菌和病毒暴露、农药、药物污染物和反复头部外伤,都已知会导致脑部炎症。活化的小胶质细胞可能特异性靶向DA神经元的机制仍然是证明活化的小胶质细胞在PD中起致病作用的关键缺失环节。然而,如果能够建立这样的联系,那么在PD中使用抑制小胶质细胞激活的药物进行临床干预试验可能是有必要的。