Methods of treating or suppressing mitochondrial diseases, such as Friedreich's ataxia (FRDA), Leber's Hereditary Optic Neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactacidosis, stroke (MELAS), or Kearns-Sayre Syndrome (KSS) are disclosed, as well as compounds useful in the methods of the invention. Energy biomarkers useful in assessing the metabolic state of a subject and the efficacy of treatment are also disclosed.
本发明公开了治疗或抑制线粒体疾病的方法,例如弗里德雷希共济失调症(FR
DA)、勒伯遗传性视神经病变(LHON)、线粒体肌病、脑病、
乳酸中毒、中风(ME
LAS)或科恩斯-萨耶综合症(KSS),以及在本发明方法中有用的化合物。本发明还公开了评估受试者代谢状态和治疗效果有用的能量
生物标志物。