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Abstract：
Neuro-muscular disorders include a variety of diseases induced by genetic mutations resulting in muscle weakness and waste, swallowing and breathing difficulties. However, muscle alterations and nerve depletions involve specific molecular and cellular mechanisms which lead to the loss of motor-nerve or skeletal-muscle function, often due to an excessive cell death. Morphological and molecular studies demonstrated that a high number of these disorders seem characterized by an upregulated apoptosis which significantly contributes to the pathology. Cell death involvement is the consequence of some cellular processes that occur during diseases, including mitochondrial dysfunction, protein aggregation, free radical generation, excitotoxicity and inflammation. The latter represents an important mediator of disease progression, which, in the central nervous system, is known as neuroinflammation, characterized by reactive microglia and astroglia, as well the infiltration of peripheral monocytes and lymphocytes. Some of the mechanisms underlying inflammation have been linked to reactive oxygen species accumulation, which trigger mitochondrial genomic and respiratory chain instability, autophagy impairment and finally neuron or muscle cell death. This review discusses the main inflammatory pathways contributing to cell death in neuro-muscular disorders by highlighting the main mechanisms, the knowledge of which appears essential in developing therapeutic strategies to prevent the consequent neuron loss and muscle wasting.
摘要：
神经肌肉疾病包括由基因突变引起的各种疾病，导致肌肉无力和浪费，吞咽困难和呼吸困难。然而,肌肉改变和神经衰竭涉及导致运动神经或骨骼肌功能丧失的特定分子和细胞机制，通常是由于过度的细胞死亡。形态学和分子研究表明，大量这些疾病的特征似乎是上调的细胞凋亡，这对病理学有很大贡献。细胞死亡参与是疾病期间发生的一些细胞过程的结果，包括线粒体功能障碍,蛋白质聚集，自由基的产生，兴奋毒性和炎症。后者代表了疾病进展的重要介质，which,在中枢神经系统中，被称为神经炎症，以反应性小胶质细胞和星形胶质细胞为特征，以及外周单核细胞和淋巴细胞的浸润。炎症的一些潜在机制与活性氧积累有关，引发线粒体基因组和呼吸链不稳定，自噬受损，最终导致神经元或肌肉细胞死亡。这篇综述通过强调主要机制来讨论神经肌肉疾病中导致细胞死亡的主要炎症途径。在制定治疗策略以防止随之而来的神经元丢失和肌肉萎缩方面，这方面的知识似乎是必不可少的。
