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Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain.
Doni D, Cavion F, Bortolus M, Baschiera E, Muccioli S, Tombesi G, d'Ettorre F, Ottaviani D, Marchesan E, Leanza L, Greggio E, Ziviani E, Russo A, Bellin M, Sartori G, Carbonera D, Salviati L, Costantini P. Doni D, et al. Among authors: costantini p. Cell Death Dis. 2023 Dec 8;14(12):805. doi: 10.1038/s41419-023-06320-y. Cell Death Dis. 2023. PMID: 38062036 Free PMC article.
Biophysical characterisation of the recombinant human frataxin precursor.
Castro IH, Ferrari A, Herrera MG, Noguera ME, Maso L, Benini M, Rufini A, Testi R, Costantini P, Santos J. Castro IH, et al. Among authors: costantini p. FEBS Open Bio. 2018 Jan 25;8(3):390-405. doi: 10.1002/2211-5463.12376. eCollection 2018 Mar. FEBS Open Bio. 2018. PMID: 29511616 Free PMC article.
Correction: Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain.
Doni D, Cavion F, Bortolus M, Baschiera E, Muccioli S, Tombesi G, d'Ettorre F, Ottaviani D, Marchesan E, Leanza L, Greggio E, Ziviani E, Russo A, Bellin M, Sartori G, Carbonera D, Salviati L, Costantini P. Doni D, et al. Among authors: costantini p. Cell Death Dis. 2024 Jan 29;15(1):93. doi: 10.1038/s41419-024-06459-2. Cell Death Dis. 2024. PMID: 38287008 Free PMC article. No abstract available.
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
23 results