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首页> 外文期刊>International Journal of Basic and Applied Sciences >Neuromuscular, cognitive and metabolic implications of McArdle Syndrome: a global overview
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Neuromuscular, cognitive and metabolic implications of McArdle Syndrome: a global overview

机译：McArdle综合征的神经肌肉，认知和代谢影响：全球概述

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Carbohydrates are the main source of body energy and they can be stored in the organism in form of glycogen and degraded when there is a need for energy. However, McArdle Syndrome patients exhibit problems to degrade glycogen due to a deficiency in myophosphorylase enzyme, making these patients intolerant to high intensity exercises because a lack of ATP available in muscle cells. It has been found muscular weakness and subsarcolemmal accumulation of glycogen in muscle fibers and in neuronal cells in McArdle Syndrome patients. In its later-onset form, it is associated to muscular dysmorphia, myoglobinuria and rhabdomyolisis. Analyzing the biochemical aspects, it is possible to notice that these patients have a low rate of ATP production due to a reduction in the availability of glucose, reducing oxidative phosphorylation. However, the metabolic “second wind” effect allows the use of other energy sources. Excessively decreased exercise-induced lactate is also characteristic of patients with McArdle Syndrome. Electromyography studies describe alterations in nerve conduction and the necessity of recruiting more motor units to contract muscle in these patients. McArdle Syndrome produces several metabolic changes in patients due to absence of myophophorylase activity. The practice of aerobic exercise acts positively in these patients probably by increasing mitochondrial metabolism.
机译：碳水化合物是人体能量的主要来源，它们可以糖原的形式存储在生物体中，并在需要能量时降解。但是，由于肌磷酸化酶缺乏，麦克阿德综合症患者表现出降解糖原的问题，由于肌肉细胞中缺乏可用的ATP，使这些患者不耐高强度运动。已经发现McArdle综合症患者的肌肉无力和糖原在肌纤维和神经元细胞中的肌膜下积聚。它以较晚发作的形式与肌肉畸形，肌红蛋白尿和横纹肌症相关。分析生化方面，可能会发现由于葡萄糖的可用性降低，氧化磷酸化降低，这些患者的ATP生成率较低。但是，新陈代谢的“第二次风”效应允许使用其他能源。麦克阿德综合症患者的特征还在于运动引起的乳酸过度减少。肌电图研究描述了这些患者神经传导的改变以及需要募集更多运动单位以收缩肌肉的必要性。由于缺乏肌磷酸化酶活性，麦克阿德综合症会在患者体内产生一些代谢变化。有氧运动在这些患者中可能通过增加线粒体代谢而发挥积极作用。

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