The Neuromuscular Junction: Roles in Aging and Neuromuscular Disease.
The Neuromuscular Junction: Roles in Aging and Neuromuscular Disease.
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DOI：
10.3390/ijms22158058
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发表时间：
2021-07-28
影响因子：
5.6
通讯作者：
Lovering RM
中科院分区：
文献类型：
--
作者：
Iyer SR;Shah SB;Lovering RM
神经肌肉接头（NMJ）是桥接运动神经元和骨骼肌纤维的特化突触，并且对于将源自运动神经元的电脉冲转换为肌纤维中的动作电位至关重要。对骨骼肌损伤、肌营养不良症和少肌症的影响因素的考虑不能仅限于肌肉固有的过程，因为数据显示，这些疾病会引起去神经样发现，例如NMJ形态碎片和神经肌肉传递中相应的功能变化。NMJ的原发性缺陷也会影响运动神经元疾病、先天性肌无力综合征和重症肌无力的功能丧失，导致骨骼肌无力和疲劳加剧。这些发现强调了NMJ在神经肌肉表现中的作用。不管原因或影响，功能性去神经支配现在是肌肉减少症和肌肉疾病的公认后果。在这个简短的审查，我们提供了一个概述的病理病因，症状和治疗策略相关的NMJ。特别是，我们研究了NMJ作为疾病修饰剂和神经肌肉损伤和疾病的潜在治疗靶点的作用。
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.
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