USE OF COMPOUND SS-31 IN PREPARATION OF DRUGS FOR TREATING FRIEDREICH'S ATAXIA AND RELATED DISEASES

摘要

Disclosed is the use of compound Szeto-Schiller-31 (SS-31) in the preparation of drugs for treating Friedreich's ataxia (FRDA) and related diseases. The present invention belongs to the pharmaceutical field, and particularly relates to the use of SS-31 in the treatment of the FRDA disease. The inventor of the present invention has found, through experiments, that SS-31 up-regulates the expression of the frataxin (FXN) in cells of FRDA patients at the translational level; regulates the iron metabolism in cells of FRDA patients; promotes the synthesis of iron-sulfur clusters in mitochondria; improves the mitochondrial function; reduces the production of ROS in patient's cells; and enhances the ability of cells to resist oxidative stress. The results show that SS-31 has a potential value in the treatment of FRDA.