Suh Yoo-Hun, Checler Frederic
Department of Pharmacology, College of Medicine, National Creative Research Initiative Center for Alzheimer's Dementia and Neuroscience Research Institute, MRC, Seoul National University, Seoul, South Korea.
Pharmacol Rev. 2002 Sep;54(3):469-525. doi: 10.1124/pr.54.3.469.
Alzheimer's disease (AD) is the most common cause of dementia that arises on a neuropathological background of amyloid plaques containing beta-amyloid (A beta) derived from amyloid precursor protein (APP) and tau-rich neurofibrillary tangles. To date, the cause and progression of both familial and sporadic AD have not been fully elucidated. The autosomal-dominant inherited forms of early-onset Alzheimer's disease are caused by mutations in the genes encoding APP, presenilin-1 (chromosome 14), and presenilin-2 (chromosome 1). APP is processed by several different proteases such as secretases and/or caspases to yield A beta and carboxyl-terminal fragments, which have been implicated in the pathogenesis of Alzheimer's disease. Alzheimer's disease and Parkinson's disease are associated with the cerebral accumulation of A beta and alpha-synuclein, respectively. Some patients have clinical and pathological features of both diseases, raising the possibility of overlapping pathogenic pathways. Recent studies have strongly suggested the possible pathogenic interactions between A beta, presenilins, and/or alpha-synuclein. Therefore, treatments that block the accumulation of A beta and alpha-synuclein might benefit a broad spectrum of neurodegenerative disorders. This review covers the trafficking and processing of APP, amyloid cascade hypothesis in AD pathogenesis, physiological and pathological roles of presenilins, molecular characteristics of alpha-synuclein, their interactions, and therapeutic strategies for AD.
阿尔茨海默病(AD)是痴呆最常见的病因,其神经病理学背景是含有源自淀粉样前体蛋白(APP)的β淀粉样蛋白(Aβ)的淀粉样斑块和富含tau的神经原纤维缠结。迄今为止,家族性和散发性AD的病因及进展尚未完全阐明。早发性阿尔茨海默病的常染色体显性遗传形式是由编码APP、早老素-1(14号染色体)和早老素-2(1号染色体)的基因突变引起的。APP由几种不同的蛋白酶如分泌酶和/或半胱天冬酶加工,产生Aβ和羧基末端片段,这些片段与阿尔茨海默病的发病机制有关。阿尔茨海默病和帕金森病分别与Aβ和α-突触核蛋白在大脑中的积累有关。一些患者具有这两种疾病的临床和病理特征,这增加了致病途径重叠的可能性。最近的研究强烈提示了Aβ、早老素和/或α-突触核蛋白之间可能存在的致病相互作用。因此,阻止Aβ和α-突触核蛋白积累的治疗方法可能对广泛的神经退行性疾病有益。本综述涵盖了APP的运输和加工、AD发病机制中的淀粉样蛋白级联假说、早老素的生理和病理作用、α-突触核蛋白的分子特征、它们之间的相互作用以及AD的治疗策略。