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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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Detalhes bibliográficos
Publicado no:Cell Rep
Main Authors: Benini, Monica, Fortuni, Silvia, Condò, Ivano, Alfedi, Giulia, Malisan, Florence, Toschi, Nicola, Serio, Dario, Massaro, Damiano Sergio, Arcuri, Gaetano, Testi, Roberto, Rufini, Alessandra
Formato: Artigo
Idioma:Inglês
Publicado em: Cell Press 2017
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5329121/
https://ncbi.nlm.nih.gov/pubmed/28228265
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.celrep.2017.01.079
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