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

Friedreich's ataxia (FRDA) is the most common hereditary ataxia, affecting ∼3 in 100 000 individuals in Caucasian populations. It is caused by intronic GAA repeat expansions that hinder the expression of the FXN gene, resulting in defective levels of the mitochondrial protein frataxin. Sensory...

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Autors principals: Tomassini, Barbara, Arcuri, Gaetano, Fortuni, Silvia, Sandi, Chiranjeevi, Ezzatizadeh, Vahid, Casali, Carlo, Condò, Ivano, Malisan, Florence, Al-Mahdawi, Sahar, Pook, Mark, Testi, Roberto
Format: Artigo
Idioma:Inglês
Publicat: Oxford University Press 2012
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Articles
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3373236/
https://ncbi.nlm.nih.gov/pubmed/22447512
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/dds110
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