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E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia
Friedreich ataxia (FRDA) is a severe genetic neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin. To date, there is no therapy to treat this condition. The amount of residual frataxin critically affects the severity of the disease; thus, attempts to restore p...
Autores principales: | Benini, Monica, Fortuni, Silvia, Condò, Ivano, Alfedi, Giulia, Malisan, Florence, Toschi, Nicola, Serio, Dario, Massaro, Damiano Sergio, Arcuri, Gaetano, Testi, Roberto, Rufini, Alessandra |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cell Press
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5329121/ https://www.ncbi.nlm.nih.gov/pubmed/28228265 http://dx.doi.org/10.1016/j.celrep.2017.01.079 |
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