Autonomic dysfunction in muscular dystrophy: a theoretical framework for muscle reflex involvement.
Autonomic dysfunction in muscular dystrophy: a theoretical framework for muscle reflex involvement.
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DOI：
10.3389/fphys.2014.00047
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发表时间：
2014
影响因子：
4
通讯作者：
Mizuno M
中科院分区：
文献类型：
--
作者：
Smith SA;Downey RM;Williamson JW;Mizuno M
肌营养不良是一组异质性遗传性疾病，其最突出的临床特征是骨骼肌的进行性变性。在几种形式的疾病中，心肌的功能也受到影响。这组疾病的主要缺陷是由对细胞结构和/或性能重要的肌细胞蛋白质突变引起的。话虽如此，越来越多的证据表明，自主神经功能障碍的发展可能是继发性的骨骼和心肌病的肌肉萎缩症的产生。事实上，交感神经和副交感神经活动的调节异常已在许多肌营养不良变体中报道。然而，介导这种自主神经功能障碍的机制仍然相对未知。运动加压反射是一种起源于骨骼肌的自主反射，已知其在受到刺激时对交感神经和副交感神经活动的控制有显著贡献。鉴于骨骼肌病变与肌营养不良症的发展，这是合乎逻辑的建议，这种反射的功能也可能是异常的疾病的发病机制。因此，它可能会导致或加剧表现出的自主神经功能障碍。这种可能性沿着的基本描述运动加压反射功能在健康和疾病的审查。更好地了解可能导致肌营养不良症自主神经功能障碍的机制，不仅可以促进进一步的研究，而且还可能导致确定新的治疗靶点治疗肌营养不良症。
Muscular dystrophies are a heterogeneous group of genetically inherited disorders whose most prominent clinical feature is progressive degeneration of skeletal muscle. In several forms of the disease, the function of cardiac muscle is likewise affected. The primary defect in this group of diseases is caused by mutations in myocyte proteins important to cellular structure and/or performance. That being stated, a growing body of evidence suggests that the development of autonomic dysfunction may secondarily contribute to the generation of skeletal and cardio-myopathy in muscular dystrophy. Indeed, abnormalities in the regulation of both sympathetic and parasympathetic nerve activity have been reported in a number of muscular dystrophy variants. However, the mechanisms mediating this autonomic dysfunction remain relatively unknown. An autonomic reflex originating in skeletal muscle, the exercise pressor reflex, is known to contribute significantly to the control of sympathetic and parasympathetic activity when stimulated. Given the skeletal myopathy that develops with muscular dystrophy, it is logical to suggest that the function of this reflex might also be abnormal with the pathogenesis of disease. As such, it may contribute to or exacerbate the autonomic dysfunction that manifests. This possibility along with a basic description of exercise pressor reflex function in health and disease are reviewed. A better understanding of the mechanisms that possibly underlie autonomic dysfunction in muscular dystrophy may not only facilitate further research but could also lead to the identification of new therapeutic targets for the treatment of muscular dystrophy.
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