Armijo Enrique, Gonzalez Cesar, Shahnawaz Mohammad, Flores Andrea, Davis Brian, Soto Claudio
Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, TX 77030, USA; Universidad de los Andes, Facultad de Medicina, Av. San Carlos de Apoquindo 2200, Las Condes, Santiago, Chile.
Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, TX 77030, USA.
Neurosci Lett. 2017 Feb 3;639:74-81. doi: 10.1016/j.neulet.2016.12.060. Epub 2016 Dec 26.
Alzheimer's disease (AD) is the most common cause of late-life dementia and represents one of the leading causes of death worldwide. The generation of induced pluripotent stem cells (iPSC) has facilitated the production and differentiation of stem cells from patients somatic cells, offering new opportunities to model AD and other diseases in vitro. In this study, we generated iPSCs from skin fibroblasts obtained from a healthy individual, as well as sporadic (sAD) and familial AD (fAD, PSEN1-A246E mutation) patients. iPSC lines were differentiated into neuronal precursors (iPSC-NPCs) and neurons that were subjected to amyloid beta (Aβ) toxicity assays. We found that neurons derived from the fAD patient have a higher susceptibility to Aβ1-42 oligomers compared with neurons coming from healthy and sAD individuals. Our findings suggest that neurons from patients with PSEN1-A246E mutation have intrinsic properties that make them more susceptible to the toxic effects of Aβ1-42 oligomers in the AD brain.
阿尔茨海默病(AD)是晚年痴呆最常见的病因,也是全球主要死因之一。诱导多能干细胞(iPSC)的产生推动了从患者体细胞生成和分化干细胞,为在体外模拟AD及其他疾病提供了新机遇。在本研究中,我们从一名健康个体以及散发型(sAD)和家族性AD(fAD,PSEN1 - A246E突变)患者获取的皮肤成纤维细胞中生成了iPSC。将iPSC系分化为神经元前体细胞(iPSC - NPCs)和神经元,并对其进行β淀粉样蛋白(Aβ)毒性检测。我们发现,与来自健康个体和sAD个体的神经元相比,源自fAD患者的神经元对Aβ1 - 42寡聚体具有更高的敏感性。我们的研究结果表明,携带PSEN1 - A246E突变患者的神经元具有内在特性,使其在AD大脑中更易受到Aβ1 - 42寡聚体毒性作用的影响。