Institute of Neuroscience, Newcastle University, Newcastle upon Tyne NE1 7RU, UK.
Int J Mol Sci. 2017 Oct 19;18(10):2183. doi: 10.3390/ijms18102183.
The commands that control animal movement are transmitted from motor neurons to their target muscle cells at the neuromuscular junctions (NMJs). The NMJs contain many protein species whose role in transmission depends not only on their inherent properties, but also on how they are distributed within the complex structure of the motor nerve terminal and the postsynaptic muscle membrane. These molecules mediate evoked chemical transmitter release from the nerve and the action of that transmitter on the muscle. Human NMJs are among the smallest known and release the smallest number of transmitter "quanta". By contrast, they have the most deeply infolded postsynaptic membranes, which help to amplify transmitter action. The same structural features that distinguish human NMJs make them particularly susceptible to pathological processes. While much has been learned about the molecules which mediate transmitter release and action, little is known about the molecular processes that control the growth of the cellular and subcellular components of the NMJ so as to give rise to its mature form. A major challenge for molecular biologists is to understand the molecular basis for the development and maintenance of functionally important aspects of NMJ structure, and thereby to point to new directions for treatment of diseases in which neuromuscular transmission is impaired.
控制动物运动的指令是由运动神经元传递到它们在神经肌肉接头 (NMJ) 的目标肌肉细胞的。NMJ 包含许多蛋白质种类，其在传递中的作用不仅取决于它们的固有特性，还取决于它们在运动神经末梢和突触后肌肉膜的复杂结构中的分布方式。这些分子介导神经释放诱发的化学递质和递质对肌肉的作用。人类 NMJ 是已知的最小的 NMJ 之一，释放的递质“量子”数量最少。相比之下，它们具有最深的折叠突触后膜，这有助于放大递质的作用。区分人类 NMJ 的相同结构特征使它们特别容易受到病理过程的影响。虽然已经了解了介导递质释放和作用的分子，但对于控制 NMJ 细胞和亚细胞成分生长的分子过程知之甚少，这些过程导致 NMJ 的成熟形式。分子生物学家面临的主要挑战是理解 NMJ 结构中具有功能重要性的方面的发育和维持的分子基础，从而为治疗神经肌肉传递受损的疾病指明新的方向。
