Preventing the ubiquitin–proteasome-dependent degradation of frataxin, the protein defective in Friedreich's ataxia

By Rufini Alessandra   Fortuni Silvia   Arcuri Gaetano   Condò Ivano   Serio Dario   Incani Ottaviano   Malisan Florence   Ventura Natascia   Testi Roberto  

Human Molecular Genetics, Vol. 20, Iss. 7, 2011-04 ,pp. : 1253-1261

Oxford University Press

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Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia

By Benada O.   Cavadini P.   O’Neill H.A.   Isaya G.  

Human Molecular Genetics, Vol. 11, Iss. 3, 2002-02 ,pp. : 217-227

Oxford University Press

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Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model

By Tomassini Barbara   Arcuri Gaetano   Fortuni Silvia   Sandi Chiranjeevi   Ezzatizadeh Vahid   Casali Carlo   Condò Ivano   Malisan Florence   Al-Mahdawi Sahar   Pook Mark   Testi Roberto  

Human Molecular Genetics, Vol. 21, Iss. 13, 2012-07 ,pp. : 2855-2861

Oxford University Press

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Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia

By Seznec Hervé   Simon Delphine   Monassier Laurent   Criqui-Filipe Paola   Gansmuller Anne   Rustin Pierre   Koenig Michel   Puccio Hélène  

Human Molecular Genetics, Vol. 13, Iss. 10, 2004-05 ,pp. : 1017-1024

Oxford University Press

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Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia

By Auchre Franoise   Santos Renata   Planamente Sara   Lesuisse Emmanuel   Camadro Jean-Michel  

Human Molecular Genetics, Vol. 17, Iss. 18, 2008-09 ,pp. : 2790-2802

Oxford University Press

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