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The displacement of frataxin from the mitochondrial cristae correlates with abnormal respiratory supercomplexes formation and bioenergetic defects in cells of Friedreich ataxia patients.
Doni D, Rigoni G, Palumbo E, Baschiera E, Peruzzo R, De Rosa E, Caicci F, Passerini L, Bettio D, Russo A, Szabò I, Soriano ME, Salviati L, Costantini P. Doni D, et al. Among authors: passerini l. FASEB J. 2021 Mar;35(3):e21362. doi: 10.1096/fj.202000524RR. FASEB J. 2021. PMID: 33629768
Effects of Fe2+/Fe3+ Binding to Human Frataxin and Its D122Y Variant, as Revealed by Site-Directed Spin Labeling (SDSL) EPR Complemented by Fluorescence and Circular Dichroism Spectroscopies.
Doni D, Passerini L, Audran G, Marque SRA, Schulz M, Santos J, Costantini P, Bortolus M, Carbonera D. Doni D, et al. Among authors: passerini l. Int J Mol Sci. 2020 Dec 17;21(24):9619. doi: 10.3390/ijms21249619. Int J Mol Sci. 2020. PMID: 33348670 Free PMC article.