abstract |
PROBLEM TO BE SOLVED: To provide a 4- (p-quinolyl) -2-hydroxybutaneamide derivative for treating mitochondrial disease. Treatment of mitochondrial diseases such as Friedreich's ataxia (FRDA), Leber's hereditary optic neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactoacidosis, and stroke (MELAS), Kearns-Sayer syndrome (KSS). Alternatively, methods of inhibition are disclosed, as well as compounds useful in the methods of the invention, such as 4- (p-quinolyl) -2-hydroxybutaneamide derivatives. Also disclosed are methods and compounds useful in treating other disorders such as amyotrophic lateral sclerosis (ALS), Huntington's disease, Parkinson's disease, and pervasive developmental disorders such as autism. Energy biomarkers useful in assessing a subject's metabolic status and therapeutic efficacy are also disclosed. [Selection diagram] None |