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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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Shranjeno v:
Bibliografske podrobnosti
izdano v: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
Format: Artigo
Jezik:Inglês
Izdano: Cell Press 2017
Teme:
Online dostop: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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