Abstract：
Located between skeletal muscle fibers and motoneurons, the neuromuscular junction is a chemical synapse essential for the transmission of information from nervous system to skeletal muscle. There are many diseases related to neuromuscular junction dysfunction, including myasthenia gravis, Lambert‑Eaton myasthenic syndrome, congenital myasthenic syndromes, amyotrophic lateral sclerosis, and spinal muscular atrophy. The pathophysiological mechanisms of these diseases have been investigated using many animal models. Among them, mouse models are the most commonly used and have provided the majority of current data. Moreover, advances in human induced pluripotent stem cell technology has resulted in new opportunities to study neuromuscular junction disorders from both patients and healthy individuals. Currently, patient‑specific induced pluripotent stem cells derived from motor neurons have begun to be studied. These studies will help us achieve a more comprehensive understanding of diseases related to neuromuscular junction disorders. We will describe the research models of neuromuscular junction disorders and provide an overview of recent key findings.
摘要：
位于骨骼肌纤维和运动神经元之间，神经肌肉接头是一种化学突触，对神经系统向骨骼肌传递信息至关重要。有许多与神经肌肉接头功能障碍有关的疾病，包括重症肌无力,Lambert-Eaton肌无力综合征,先天性肌无力综合征,肌萎缩侧索硬化，和脊髓性肌萎缩症.已经使用许多动物模型研究了这些疾病的病理生理机制。其中,小鼠模型是最常用的，并且提供了大多数当前数据。此外，人类诱导多能干细胞技术的进步为研究患者和健康个体的神经肌肉接头疾病提供了新的机会。目前,来自运动神经元的患者特异性诱导多能干细胞已经开始研究.这些研究将帮助我们更全面地了解与神经肌肉接头疾病相关的疾病。我们将描述神经肌肉接头疾病的研究模型，并提供最近的关键发现的概述。
