| abstract |
Disclosed are methods of treating or suppressing mitochondrial diseases such as Friedreich's ataxia (FRDA), Leber hereditary optic neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactic acidosis and stroke (MELAS), Kearns-Sayre syndrome (KSS); as well as compounds useful in the methods of the present invention, for example, 4- (p-quinolyl) -2-hydroxybutanamide derivatives. Also disclosed are methods and compounds useful for treating other disorders such as amyotrophic lateral sclerosis (ALS), Huntington's disease, Parkinson's disease, and a general developmental disorder such as autism. Also disclosed are energy biomarkers useful for assessing the metabolic state of a subject and the effectiveness of treatment. Also disclosed are methods for modulating, normalizing, or enhancing energy biomarkers, as well as compounds useful for these methods. |