Frataxin Silencing Inactivates Mitochondrial Complex I in NSC34 Motoneuronal Cells and Alters Glutathione Homeostasis

Friedreich’s ataxia (FRDA) is a hereditary neurodegenerative disease characterized by a reduced synthesis of the mitochondrial iron chaperon protein frataxin as a result of a large GAA triplet-repeat expansion within the first intron of the frataxin gene. Despite neurodegeneration being the prominen...

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Bibliografiske detaljer
Main Authors: Carletti, Barbara, Piermarini, Emanuela, Tozzi, Giulia, Travaglini, Lorena, Torraco, Alessandra, Pastore, Anna, Sparaco, Marco, Petrillo, Sara, Carrozzo, Rosalba, Bertini, Enrico, Piemonte, Fiorella
Format: Artigo
Sprog:Inglês
Udgivet: Molecular Diversity Preservation International (MDPI) 2014
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4013596/
https://ncbi.nlm.nih.gov/pubmed/24714088
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms15045789
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