重症肌无力中的线粒体功能

Mitochondrial function in myasthenia gravis.

作者信息

Lousa M, Gobernado J M, Gimeno A, Gosalvez M

出版信息

Eur Neurol. 1983;22(1):53-7. doi: 10.1159/000115536.

Abstract

We determined the respiration rate, respiratory control and ADP/O ratios, with different substrates in mitochondria isolated from 2 patients with myasthenia gravis and compared them with normal human muscle. In all cases studied, a severe alteration of the respiratory control with variable derangement of oxidative phosphorylation was found. This abnormality of mitochondrial metabolism was referred to by Luft as 'loosely coupled' and was reported in other neuromuscular diseases. In our opinion this defective oxidative metabolism is nonspecific.

摘要

我们测定了从2例重症肌无力患者分离出的线粒体在使用不同底物时的呼吸速率、呼吸控制及ADP/O比值，并将其与正常人体肌肉进行比较。在所研究的所有病例中，均发现呼吸控制严重改变，氧化磷酸化出现不同程度的紊乱。这种线粒体代谢异常被卢夫特称为“松散偶联”，在其他神经肌肉疾病中也有报道。我们认为这种氧化代谢缺陷是非特异性的。