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

Hum. Mol. Genet. (2012) doi: 10.1093/hmg/dds110 First published online: March 23, 2012

Barbara Tomassini1, Gaetano Arcuri1, Silvia Fortuni1, Chiranjeevi Sandi2, Vahid
Ezzatizadeh2, Carlo Casali3, Ivano Condò1, Florence Malisan1, Sahar Al-Mahdawi2,
Mark Pook2 and Roberto Testi1

1 Laboratory of Immunology and Signal Transduction, University of Rome “Tor
Vergata”, 00133 Rome, Italy
2 Division of Biosciences, School of Health Sciences and Social Care, Brunel University,
Uxbridge, UB8 3PH, UK
3 Department of Neurology, University of Rome “La Sapienza”, Polo Pontino, 04100
Latina, Italy

Importantly,in vivo treatment with IFN increases frataxin expression in DRG neurons, prevents their pathological changes and ameliorates the sensorimotor performance in FRDA mice. These results disclose new roles for IFN in cellular metabolism and have direct implications for the treatment of FRDA.


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