Muscle energy metabolism in human phosphofructokinase deficiency as recorded by 31P nuclear magnetic resonance spectroscopy
Muscle energy metabolism in human phosphofructokinase deficiency as recorded by 31P nuclear magnetic resonance spectroscopy
复制标题
31P核磁共振波谱记录的人磷酸果糖激酶缺乏症的肌肉能量代谢
作者：
Z. Argov;W. Bank;John Marchis;J. Leigh;B. Chance
对一例肌肉磷酸果糖激酶缺乏症患者的31P核磁共振研究发现：首先，ATP代谢在休息和运动时受到干扰。在休息时，ATP水平低于正常水平，并在运动中继续下降。其次，运动动力学正常，表明线粒体燃料供应正常，尽管糖酵解被阻断。第三，在长时间的低水平运动中没有观察到“磷酸盐捕获”。第四，运动后的恢复会因磷酸糖的缓慢去磷酸化而异常延长，其体内半衰期约为9分钟。这些发现证明了肌肉组织如何适应主要生物能量途径的阻断。
31P nuclear magnetic resonance studies of a patient with phosphofructokinase deficiency in muscle provided the following new findings: First, ATP metabolism is disturbed at rest and during exercise. At rest, ATP levels are lower than normal and continue to decline during exercise. Second, exercise kinetics are normal, suggesting a normal mitochondrial fuel supply although glycolysis is blocked. Third, no “phosphate trapping” is observed during prolonged low‐level exercise. Fourth, postexercise recovery is abnormally prolonged by the slow dephosphorylation of sugar phosphates, which has an in vivo half‐life of about nine minutes. These findings demonstrate how musclé tissue adapts to a block in a major bioenergetic pathway.
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
发表时间：
1985
期刊：
Journal of applied physiology (Bethesda, Md. : 1985)
影响因子：
--
作者：
Lewis,SF;Haller,RG;Cook,JD;Nunnally,RL
通讯作者：
Nunnally,RL
影响因子：
37.8
作者：
Chance,B;Clark,BJ;Nioka,S;Subramanian,H;Maris,JM;Argov,Z;Bode,H
通讯作者：
Bode,H
影响因子：
2.4
作者：
Servidei,S;DiMauro,S
通讯作者：
DiMauro,S