The Neuromuscular Junction: Roles in Aging and Neuromuscular Disease

International Journal of Molecular Sciences

Shama R. Iyer¹  Richard M. Lovering¹  Sameer B. Shah² 

[1] Department of Orthopaedics, University of Maryland School of Medicine, AHB, Room 540, 100 Penn St., Baltimore, MD 21201, USA;Departments of Orthopaedic Surgery and Bioengineering, University of California San Diego, La Jolla, CA 92093, USA;

关键词: NMJ;    muscular dystrophy;    sarcopenia;    myasthenia gravis;    exercise;   

DOI  :  10.3390/ijms22158058

来源: DOAJ

【 摘 要 】

The neuromuscular junction (NMJ) is a specialized synapse that bridges the motor neuron and the skeletal muscle fiber and is crucial for conversion of electrical impulses originating in the motor neuron to action potentials in the muscle fiber. The consideration of contributing factors to skeletal muscle injury, muscular dystrophy and sarcopenia cannot be restricted only to processes intrinsic to the muscle, as data show that these conditions incur denervation-like findings, such as fragmented NMJ morphology and corresponding functional changes in neuromuscular transmission. Primary defects in the NMJ also influence functional loss in motor neuron disease, congenital myasthenic syndromes and myasthenia gravis, resulting in skeletal muscle weakness and heightened fatigue. Such findings underscore the role that the NMJ plays in neuromuscular performance. Regardless of cause or effect, functional denervation is now an accepted consequence of sarcopenia and muscle disease. In this short review, we provide an overview of the pathologic etiology, symptoms, and therapeutic strategies related to the NMJ. In particular, we examine the role of the NMJ as a disease modifier and a potential therapeutic target in neuromuscular injury and disease.

【 授权许可】

Unknown