Motor neuron loss or degeneration is the typical characteristic of amyotrophic lateral sclerosis (ALS), which often leads to weakness, paralysis, or even death. The underlying mechanisms of motor neuron degeneration and ALS progression remain elusive, and there is no effective treatment for ALS. The advances of stem cells and reprogramming techniques has made it possible to generate patient-specific motor neurons as cell models for studying disease mechanisms and drug discovery. This review comprehensively discusses recent approaches to generate motor neurons from stem cells and somatic cells and highlights the application of induced motor neurons to modeling ALS diseases, dissecting the pathogenesis, and screening new drugs. New perspectives are also discussed on generating patient-specific motor neuron subtypes that are affected by ALS or creating 3D spinal cord organoid models for better recapitulating and understanding ALS.

中文翻译：

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特定于患者的细胞，用于建模和解码肌萎缩性侧索硬化症：进展和挑战。

运动神经元丢失或变性是肌萎缩性侧索硬化症（ALS）的典型特征，通常会导致肌无力，瘫痪甚至死亡。运动神经元变性和ALS进展的基本机制仍然难以捉摸，并且没有有效的ALS治疗方法。干细胞和重编程技术的进步使得有可能产生患者特定的运动神经元作为研究疾病机理和药物发现的细胞模型。这篇综述全面讨论了从干细胞和体细胞产生运动神经元的最新方法，并着重介绍了诱导的运动神经元在模拟ALS疾病，解剖发病机理和筛选新药方面的应用。