神経筋接合部（NMJ）の形成・維持シグナルとNMJ標的治療の開発

Molecular Mechanisms Underlying the Formation and Maintenance of Neuromuscular Junctions and Development of NMJ-targeted Therapeutics

骨格筋は個体の運動機能，認知機能や多様な臓器の健全性に必須の器官であり，骨格筋機能を支える筋収縮は運動神経による厳密な制御を受ける。運動神経軸索末端と筋管（筋線維）を結ぶ神経筋接合部（neuromuscular junction：NMJ）は骨格筋収縮の運動神経支配に必須の化学シナプスであり，その異常は筋力低下を含む多様な骨格筋機能の異常を引き起こす。本稿では骨格筋におけるNMJ形成・維持シグナルの理解とNMJ標的治療開発の概要を紹介する。

Abstract

Skeletal muscle is an essential organ for the motor functions and its defects are associated with functional impairments of other organs including brain. Muscle contraction is the fundamental skeletal muscle function and is strictly controlled by motor neuron, which requires neuromuscular junction (NMJ), a chemical synapse between motor nerve terminal and myotube (myofiber). Defects in NMJ cause various functional abnormalities of skeletal muscle including muscle weakness. This review presents an overview of the current understanding of signaling in NMJ formation and maintenance in skeletal muscle and the development of NMJ-targeted therapeutics.