Cell Therapy and Regenerative Medicine Research Center, Endocrinology and Metabolism Molecular-Cellular Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.
Proteomics Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Methods Mol Biol. 2024;2736:139-150. doi: 10.1007/7651_2022_474.
Amyotrophic lateral sclerosis (ALS) is a progressive and degenerative disorder of the nervous system that can significantly reduce the physical activity of patients at the end stages. As the field of disease pathophysiology has advanced in recent years, studies have looked at the role of neuromuscular junction's dysfunctionality in ALS. In the past years, various in vitro and in vivo models were developed to scrutinize the underlying mechanisms of the disease and investigate the effects of candidate drugs, but the application of the developed models faced many challenges. Hence, the attentions shifted to cutting-edge technologies such as the organ-on-a-chip, which can mimic the pathophysiology of the disease as a special biological platform using patient-derived cells in the integration of engineering sciences to expand researchers' perspectives on the disease. In addition, organ-on-a-chip technology can reduce some of the challenges of using other in vitro and in vivo models, which can pave the way for other discoveries and advances in this disease.
肌萎缩侧索硬化症（ALS）是一种进行性和退行性的神经系统疾病，在疾病晚期会显著降低患者的身体活动能力。近年来，随着疾病病理生理学领域的发展，研究已经关注到神经肌肉接头功能障碍在 ALS 中的作用。在过去的几年中，已经开发了各种体外和体内模型来仔细研究疾病的潜在机制，并研究候选药物的效果，但开发模型的应用面临着许多挑战。因此，人们的注意力转向了前沿技术，如器官芯片，它可以使用患者来源的细胞作为特殊的生物平台，整合工程科学来模拟疾病的病理生理学，从而扩展研究人员对疾病的认识。此外，器官芯片技术可以减少使用其他体外和体内模型的一些挑战，为该疾病的其他发现和进展铺平道路。
