Mattson Mark P
Laboratory of Neurosciences, National Institute on Aging Gerontology Research Center, Baltimore, Maryland, USA.
J Neurovirol. 2002 Dec;8(6):539-50. doi: 10.1080/13550280290100978.
Although Alzheimer's disease (AD) may not involve a transmissible agent, it does involve a pathogenic process similar to that of transmissible prion disorders (both involve a protein that adopts an abnormal pathogenic conformation in which it self-aggregates, forming amyloid deposits in and surrounding neurons) and viral dementias such as human immunodeficiency virus (HIV) encephalitis. The clinical presentation of patients with AD is dominated by cognitive deficits and emotional disturbances that result from dysfunction and degeneration of neurons in the limbic system and cerebral cortex. The pathogenic process in the brain involves deposition of insoluble aggregates of amyloid beta-peptide, oxidative stress and calcium dysregulation in neurons, and activation of inflammatory cytokine cascades involving microglia. However, AD patients also exhibit alterations in immune function. Studies of lymphocytes and lymphoblast cell lines from AD patients and age-matched normal control patients have documented alterations in cytokine and calcium signaling and increased levels of oxidative stress in immune cells from the AD patients. Studies of the pathogenic actions of mutations in presenilins and amyloid precursor protein that cause early-onset familial AD have established central roles for perturbed cellular calcium homeostasis and oxidative stress in the neurodegenerative process. Presenilin and amyloid precursor protein (APP) mutations also increase oxidative stress and perturb calcium signaling in lymphocytes in ways that alter their production of cytokines that are critical for proper immune responses. Immune dysfunction occurs prior to clinical symptoms in mouse models of AD, and brain cytokine responses to immune challenge are altered in presenilin mutant mice, suggesting a causal role for altered immune function in the disease process. Interestingly, immunization of AD mice with amyloid beta-peptide can stimulate the immune system to remove amyloid from the brain and can ameliorate memory deficits, suggesting that it may be possible to prevent AD by bolstering immune function.
尽管阿尔茨海默病(AD)可能不涉及可传播因子,但它确实涉及一种与可传播朊病毒疾病类似的致病过程(两者都涉及一种蛋白质,该蛋白质呈现异常的致病构象,在这种构象中它会自我聚集,在神经元内部和周围形成淀粉样沉积物)以及病毒性痴呆,如人类免疫缺陷病毒(HIV)脑炎。AD患者的临床表现主要由边缘系统和大脑皮层神经元功能障碍和退化导致的认知缺陷和情绪障碍所主导。大脑中的致病过程涉及β-淀粉样肽不溶性聚集体的沉积、神经元中的氧化应激和钙调节异常,以及涉及小胶质细胞的炎症细胞因子级联反应的激活。然而,AD患者也表现出免疫功能的改变。对AD患者和年龄匹配的正常对照患者的淋巴细胞和淋巴母细胞系的研究记录了细胞因子和钙信号的改变以及AD患者免疫细胞中氧化应激水平的升高。对导致早发性家族性AD的早老素和淀粉样前体蛋白突变的致病作用的研究已经确定细胞钙稳态紊乱和氧化应激在神经退行性过程中起核心作用。早老素和淀粉样前体蛋白(APP)突变还以改变对适当免疫反应至关重要的细胞因子产生的方式增加淋巴细胞中的氧化应激并扰乱钙信号。在AD小鼠模型中,免疫功能障碍在临床症状出现之前就已发生,并且早老素突变小鼠对免疫挑战的脑内细胞因子反应发生改变,这表明免疫功能改变在疾病过程中起因果作用。有趣的是,用β-淀粉样肽对AD小鼠进行免疫接种可以刺激免疫系统从大脑中清除淀粉样蛋白,并可以改善记忆缺陷,这表明通过增强免疫功能可能预防AD。