Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency

Meisel, J.D., Joshi, P.R., Spelbring, A.N. et al. Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency. Nature (2025). doi:10.1038/s41586-025-09821-2 

We show that lowering levels of wild-type FDX2 through loss of one gene copy can ameliorate the growth of frataxin mutant C. elegans or the ataxia phenotype of a mouse model of Friedreich’s ataxia under normoxic conditions. These genetic and biochemical studies indicate that restoring the stoichiometric balance of frataxin and FDX2 through partial knockdown of FDX2 may be a potential therapy for Friedreich’s ataxia.