| abstract |
The invention provides methods for treating or suppressing mitochondrial diseases such as Friedreich's ataxia (FRDA), Leber's hereditary optic neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactic acidosis and stroke (MELAS), Kearns-Sair 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 disorder of psychological development, such as autism. Energy biomarkers applicable for assessing the metabolic state of the subject and the effectiveness of treatment are also disclosed. Also disclosed are methods for modulating, normalizing or enhancing energy biomarkers, as well as compounds applicable to these methods. |