Vatiquinone is a metabolite of α-tocotrienol that has antioxidant and ferroptosis inhibitory activity.1,2 It inhibits decreases in cell viability induced by the γ-glutamylcysteine synthetase inhibitor L-buthionine-(S,R)-sulfoximine in primary fibroblasts isolated from patients with the mitochondrial diseases Friedreich's ataxia or Leigh syndrome (EC50s = 24 and 30 nM, respectively).1 Vatiquinone inhibits cell death and lipid peroxidation induced by the glutathione peroxidase 4 (GPX4) inhibitor RSL3 in primary fibroblasts isolated from patients with the mitochondrial encephalopathic epilepsy disease pontocerebellar hypoplasia type 6 (PCH6; EC50s = 17.3-21.8 and 33.1-57.6 nM, respectively).2
1.Shrader, W.D., Amagata, A., Barnes, A., et al.α-Tocotrienol quinone modulates oxidative stress response and the biochemistry of agingBioorg. Med. Chem. Lett.21(12)3693-3698(2011) 2.Kahn-Kirby, A.H., Amagata, A., Maeder, C.I., et al.Targeting ferroptosis: A novel therapeutic strategy for the treatment of mitochondrial disease-related epilepsyPLoS One14(3)e0214250(2019)