iPS cells from ALS patients- towards replacing animal models for ALS

肌萎缩侧索硬化症是一种神经退行性疾病，患者的运动神经元（连接肌肉的神经）死亡，导致运动能力丧失，并影响呼吸等关键身体过程，最终导致死亡。肌萎缩性侧索硬化症的小鼠模型已被用于测试治疗干预措施，但其中许多不是健全的系统。我们建议从肌萎缩侧索硬化症患者的皮肤细胞中制造类似干细胞的细胞，我们可以从中制造运动神经元。这些运动神经元将提供一个更类似于人类疾病的模型，因此为了解疾病过程以及测试药物提供了一个很好的模型。

ALS is a form of neurodegenerative disorder in which Motor Neurons, the nerves that connect to the muscle die leading loss of movement and affect key body processes such as breathing, eventually causing death. Mouse models for ALS have been generated to test therapeutic interventions but many of these are not robust systems. We propose to make stem cell like cells from skin cells of ALS patients from which we can make Motor neurons. These motor neurons will provide a model more akin to the human disease and therefore serve a good model to understand the disease process as well as to test drugs.