Targeting frataxin deficiency in DRG neurons and fibroblasts: omaveloxolone restores metabolic and iron balance to reduce ferroptosis

Portillo-Carrasquer M, Sanz-Alcázar A, Sánchez-López B, Delaspre F, Pazos-Gil M, Oliveira-Jorge L, Castells-Roca L, Tamarit J, Ros J, Cabiscol E. Targeting frataxin deficiency in DRG neurons and fibroblasts: omaveloxolone restores metabolic and iron balance to reduce ferroptosis. Biomed Pharmacother. 2026 Feb;195:119031. doi: 10.1016/j.biopha.2026.119031. Epub 2026 Jan 23. PMID: 41579709.

Here we used frataxin-deficient DRG neurons to better understand the drug's role in these sensory neurons. Omaveloxolone improved most of the analyzed parameters, including frataxin levels, cell survival, mitochondrial respiratory activity, iron homeostasis, oxidative stress, transferrin receptor 1 and glutathione peroxidase 4 levels, as well as the GSH/GSSG ratio.