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

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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Bibliographic Details
Main Authors: Barbara Carletti, Emanuela Piermarini, Giulia Tozzi, Lorena Travaglini, Alessandra Torraco, Anna Pastore, Marco Sparaco, Sara Petrillo, Rosalba Carrozzo, Enrico Bertini, Fiorella Piemonte
Format: Article
Language:English
Published: MDPI AG 2014-04-01
Series:International Journal of Molecular Sciences
Subjects:
Friedreich’s ataxia
neurodegeneration
glutathione
oxidative stress
mitochondrial enzymes
Online Access:http://www.mdpi.com/1422-0067/15/4/5789

Internet

http://www.mdpi.com/1422-0067/15/4/5789

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