Muscle fatigue in McArdle's disease studied by 31P-NMR: effect of glucose infusion.
Muscle fatigue in McArdle's disease studied by 31P-NMR: effect of glucose infusion.
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通过 31P-NMR 研究 McArdle 病的肌肉疲劳：葡萄糖输注的影响。
DOI：
10.1152/jappl.1985.59.6.1991
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
Nunnally,RL
中科院分区：
文献类型：
--
作者：
Lewis,SF;Haller,RG;Cook,JD;Nunnally,RL
在肌肉磷酸化酶缺乏症（麦卡德尔病）中，在剧烈运动期间会出现异常快速的疲劳。增加工作肌的底物利用率可以提高运动耐力，但对肌肉能量代谢的影响尚未研究。使用磷-31核磁共振（31 P-NMR），我们检查前臂肌肉ATP，磷酸肌酸（PCr），无机磷酸盐（Pi）和pH值在McArdle患者（MP）和两名健康受试者（HS）在休息和间歇性最大努力的握力收缩控制条件下（CC）和静脉内葡萄糖输注（GI）。在CC下，MP在130秒内抓到即将发生的前臂肌肉挛缩，肌肉PCr显著下降，Pi显著升高。在GI期间，MP在更高的张力下容易地运动超过420 s，并且PCr消耗和Pi积累减弱。在HS，肌肉PCr和Pi的变化更温和，不受GI。在MP和HS中，ATP随运动变化很小或根本没有变化。结果表明，肌肉PCr和Pi水平的改变而不是ATP参与了McArdle病的肌肉疲劳和葡萄糖输注期间运动性能的改善。
In muscle phosphorylase deficiency (McArdle's disease) there is an abnormally rapid fatigue during strenuous exercise. Increasing substrate availability to working muscle can improve exercise tolerance but the effect on muscle energy metabolism has not been studied. Using phosphorus-31 nuclear magnetic resonance (31P-NMR) we examined forearm muscle ATP, phosphocreatine (PCr), inorganic phosphate (Pi) and pH in a McArdle patient (MP) and two healthy subjects (HS) at rest and during intermittent maximal effort handgrip contractions under control conditions (CC) and during intravenous glucose infusion (GI). Under CC, MP gripped to impending forearm muscle contracture in 130 s with a marked decline in muscle PCr and a dramatic elevation in Pi. During GI, MP exercised easily for greater than 420 s at higher tensions and with attenuated PCr depletion and Pi accumulation. In HS, muscle PCr and Pi changed more modestly and were not affected by GI. In MP and HS, ATP changed little or not at all with exercise. The results suggest that alterations in the levels of muscle PCr and Pi but not ATP are involved in the muscle fatigue in McArdle's disease and the improved exercise performance during glucose infusion.