Institute of Neuroscience, Department of Clinical Neurophysiology, Uppsala University Hospital, Uppsala, Sweden.
Curr Opin Pharmacol. 2012 Jun;12(3):340-6. doi: 10.1016/j.coph.2012.02.002. Epub 2012 Feb 23.
The neuromuscular junction (NMJ) is a specialized synapse between motor neurons and skeletal muscle with a complex signaling network that assures highly reliable neuromuscular transmission. Diseases of the NMJ cause skeletal muscle fatigue and include inherited and acquired disorders that affect presynaptic, intrasynaptic or postsynaptic components. Moreover, fragmentation of the NMJ contributes to sarcopenia, the loss of muscle mass during aging. Studies from recent years indicate that the formation and stabilization of NMJs differs between various muscles and that this difference affects their response under pathological conditions. This review summarizes the most important mechanisms involved in the development, maintenance and dysfunction of the NMJ and it discusses their significance in myasthenic disorders and aging and as targets for possible future treatment of NMJ dysfunction.
神经肌肉接头（NMJ）是运动神经元和骨骼肌之间的一种特殊突触，具有复杂的信号网络，可确保高度可靠的神经肌肉传递。NMJ 疾病会导致骨骼肌疲劳，包括影响突触前、突触内或突触后成分的遗传性和获得性疾病。此外，NMJ 的碎片化导致肌肉减少症，即随着年龄增长肌肉质量的丧失。近年来的研究表明，不同肌肉中的 NMJ 形成和稳定存在差异，这种差异会影响它们在病理条件下的反应。本综述总结了 NMJ 发育、维持和功能障碍中涉及的最重要的机制，并讨论了它们在重症肌无力障碍和衰老中的意义，以及作为 NMJ 功能障碍未来可能治疗靶点的意义。
