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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Detalhes bibliográficos
Publicado no: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
Formato: Artigo
Idioma:Inglês
Publicado em: Academic Press 2015
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Article
Acesso em linha: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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