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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: costantini p. FASEB J. 2021 Mar;35(3):e21362. doi: 10.1096/fj.202000524RR. FASEB J. 2021. PMID: 33629768
Exploring iron-binding to human frataxin and to selected Friedreich ataxia mutants by means of NMR and EPR spectroscopies.
Bellanda M, Maso L, Doni D, Bortolus M, De Rosa E, Lunardi F, Alfonsi A, Noguera ME, Herrera MG, Santos J, Carbonera D, Costantini P. Bellanda M, et al. Among authors: costantini p. Biochim Biophys Acta Proteins Proteom. 2019 Nov;1867(11):140254. doi: 10.1016/j.bbapap.2019.07.007. Epub 2019 Jul 22. Biochim Biophys Acta Proteins Proteom. 2019. PMID: 31344531 Free article.
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: costantini p. Int J Mol Sci. 2020 Dec 17;21(24):9619. doi: 10.3390/ijms21249619. Int J Mol Sci. 2020. PMID: 33348670 Free PMC article.
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