Bioactive fumarate improves cardiac function and expands lifespan in Friedrech’s ataxia

Salinas, L., Figueroa, F., Montgomery, C. B., Thai, P. N., Chiamvimonvat, N., Dugar, S., Sen, S., Cortopassi, G., & Dedkova, E. N. (2023). Bioactive fumarate improves cardiac function and expands lifespan in Friedrech’s ataxia. Biophysical Journal, 122(3), 96a. doi:10.1016/j.bpj.2022.11.714 

 Friedreich's ataxia (FA) is a recessive ataxia caused by reduction of mitochondrial protein, frataxin (FXN). Cardiomyopathy is the leading cause of death in FA patients due to deficient FXN expression in the heart. We have developed a novel monomethyl fumarate prodrug, IMF, which has improved pharmacokinetic profile and compared its effect to fumarate prodrug dimethyl fumarate (DMF, Tecfidera).

Bioactive fumarate improves cardiac function and expands lifespan in Friedrech’s ataxia