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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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cell Press 2017
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