Group of Brain Development and Disease, Department of Veterinary and Animal Sciences, Faculty of Medical Sciences, University of Copenhagen, Dyrlægevej 100, 1870 Frederiksberg, Denmark.
Cells. 2023 Jan 27;12(3):420. doi: 10.3390/cells12030420.
Modeling Alzheimer's disease (AD) using human-induced pluripotent stem cells (iPSCs) is a field now spanning 15 years. Developments in the field have shown a shift in using simple 2D cortical neuron models to more advanced tri-cultures and 3D cerebral organoids that recapitulate more features of the disease. This is largely due to development and optimization of new cell protocols. In this review, we highlight recent major breakthroughs in the AD field and the implications this has in modeling AD using iPSCs (AD-iPSCs). To date, AD-iPSCs have been largely used to recapitulate and study impaired amyloid precursor protein (APP) processing and tau phosphorylation in both familial and sporadic AD. AD-iPSCs have also been studied for varying neuronal and glial dysfunctions. Moreover, they have been useful for discovering new molecular mechanisms, such as identifying proteins that bridge APP processing with tau phosphorylation and for identifying molecular pathways that bridge APP processing dysfunction with impaired cholesterol biosynthesis. Perhaps the greatest use of AD-iPSCs has been in discovering compounds via drug screening, that reduce amyloid beta (Aβ) in neurons, such as the anti-inflammatory compound, cromolyn, and antiparasitic drugs, avermectins. In addition, high content screening using AD-iPSCs has led to the identification of statins that can reduce levels of phosphorylated tau (p-Tau) in neurons. Some of these compounds have made it through to testing in human clinical trials. Improvements in omic technologies including single cell RNA sequencing and proteomics as well as advances in production of iPSC-cerebral organoids and tri-cultures is likely to result in the further discovery of new drugs and treatments for AD. Some caveats remain in the field, including, long experimental conditions to create mature neurons, high costs of media that limit research capabilities, and a lack of reproducibility using current iPSC-cerebral organoid protocols. Despite these current limitations, AD-iPSCs remain an excellent cellular model for studying AD mechanisms and for drug discovery.
使用人诱导多能干细胞(iPSC)来模拟阿尔茨海默病(AD)是一个已有 15 年历史的领域。该领域的发展表明,人们已经从简单的 2D 皮质神经元模型转向更先进的三培养物和 3D 类器官,以重现更多疾病特征。这在很大程度上是由于新细胞方案的开发和优化。在这篇综述中,我们重点介绍了 AD 领域的最新重大突破,以及这对使用 iPSC(AD-iPSC)模拟 AD 的影响。迄今为止,AD-iPSC 主要用于模拟和研究家族性和散发性 AD 中淀粉样前体蛋白(APP)处理和 tau 磷酸化受损。AD-iPSC 也被用于研究各种神经元和神经胶质功能障碍。此外,它们还可用于发现新的分子机制,例如鉴定将 APP 处理与 tau 磷酸化联系起来的蛋白质,以及鉴定将 APP 处理功能障碍与胆固醇生物合成受损联系起来的分子途径。也许 AD-iPSC 的最大用途是通过药物筛选发现化合物,这些化合物可以减少神经元中的淀粉样β(Aβ),例如抗炎化合物 cromolyn 和抗寄生虫药物 avermectins。此外,使用 AD-iPSC 的高通量筛选导致发现了可以减少神经元中磷酸化 tau(p-Tau)水平的他汀类药物。其中一些化合物已通过人体临床试验进行了测试。包括单细胞 RNA 测序和蛋白质组学在内的组学技术的改进以及 iPSC-类器官和三培养物的生产的进步,可能会进一步发现用于 AD 的新药和治疗方法。该领域仍然存在一些注意事项,包括创建成熟神经元的实验条件漫长、限制研究能力的培养基成本高以及当前 iPSC-类器官方案缺乏可重复性。尽管存在这些当前的局限性,但 AD-iPSC 仍然是研究 AD 机制和药物发现的优秀细胞模型。