Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells

Friedreich ataxia is an inherited neurodegenerative disease that leads to progressive disability. There is currently no effective treatment and patients die prematurely. The underlying genetic defect leads to reduced expression of the mitochondrial protein frataxin. Frataxin insufficiency causes mit...

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Bibliographic Details
Published in:Neurobiol Dis
Main Authors: Rufini, Alessandra, Cavallo, Francesca, Condò, Ivano, Fortuni, Silvia, De Martino, Gabriella, Incani, Ottaviano, Di Venere, Almerinda, Benini, Monica, Massaro, Damiano Sergio, Arcuri, Gaetano, Serio, Dario, Malisan, Florence, Testi, Roberto
Format: Artigo
Language:Inglês
Published: Academic Press 2015
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC4358773/
https://ncbi.nlm.nih.gov/pubmed/25549872
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2014.12.011
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