Legay Claire, Mei Lin
Université Paris Descartes, CNRS UMR 8119, Sorbonne Paris Cité, Paris, France.
Department of Neurosciences and Regenerative medicine, Charlie Norwood VA Medical Center, Augusta, Georgia, USA.
J Neurochem. 2017 Aug;142 Suppl 2(Suppl 2):59-63. doi: 10.1111/jnc.14028. Epub 2017 Apr 27.
The neuromuscular junction (NMJ) is indispensable for survival. This synapse between motoneurons and skeletal muscle fibers allows posture, movement and respiration. Therefore, its dysfunction creates pathologies than can be lethal. The molecular mechanisms of NMJ development and maintenance are the subject of intensive studies. This mini-review focuses on some of the most recent discoveries. An unexpected role for a protein, rapsyn, which has been known for 40 years to aggregate acetylcholine receptors has emerged. A new cell partner at NMJ has been unmasked and is challenging our understanding of the functioning of this synapse. Toxins are now used as new tools to study degeneration/regeneration. The possibility of creating human NMJ in vitro is within reach with major consequences for drug screening. Wnts are secreted neurogenic factors that have been involved in vitro in acetylcholine receptor clustering, but their precise role in vivo remains to be clarified. All these data are raising new and exciting perspectives in the field and are discussed in this Review. This is an article for the special issue XVth International Symposium on Cholinergic Mechanisms.
神经肌肉接头(NMJ)对于生存至关重要。运动神经元与骨骼肌纤维之间的这种突触使姿势、运动和呼吸成为可能。因此,其功能障碍会引发可能致命的病症。NMJ发育和维持的分子机制是深入研究的主题。本综述聚焦于一些最新发现。一种名为rapsyn的蛋白质已出现意想不到的作用,40年来人们一直知道它能聚集乙酰胆碱受体。NMJ处一个新的细胞伙伴已被揭示,这正在挑战我们对该突触功能的理解。毒素如今被用作研究退化/再生的新工具。体外构建人类NMJ的可能性已触手可及,这对药物筛选具有重大影响。Wnts是分泌型神经源性因子,在体外已发现其参与乙酰胆碱受体聚集,但其在体内的确切作用仍有待阐明。所有这些数据都为该领域带来了新的、令人兴奋的前景,并在本综述中进行了讨论。这是一篇发表于第十五届国际胆碱能机制研讨会特刊的文章。