Thi Hong Lien Han, Jean-Michel Camadro, Florent Barbault, Renata Santos, Jean-Michel El Hage Chahine, Nguyet-Thanh Ha-Duong; Biochimica et Biophysica Acta (BBA) - General Subjects, Volume 1863, Issue 5, 2019, Pages 883-892, ISSN 0304-4165, doi:10.1016/j.bbagen.2019.02.011.
Friedreich's ataxia results from a decreased expression of the nuclear gene encoding the mitochondrial protein, frataxin. Frataxin participates in the biosynthesis of iron-sulfur clusters and heme cofactors, as well as in iron storage and protection against oxidative stress. How frataxin interacts with the antioxidant defence components is poorly understood.
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