Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain

Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain

Abstract Friedreich ataxia (FRDA) is a rare, inherited neurodegenerative disease caused by an expanded GAA repeat in the first intron of the FXN gene, leading to transcriptional silencing and reduced expression of frataxin. Frataxin participates in the mitochondrial assembly of FeS clusters, redox c...

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Bibliographic Details
Main Authors: Davide Doni, Federica Cavion, Marco Bortolus, Elisa Baschiera, Silvia Muccioli, Giulia Tombesi, Federica d’Ettorre, Daniele Ottaviani, Elena Marchesan, Luigi Leanza, Elisa Greggio, Elena Ziviani, Antonella Russo, Milena Bellin, Geppo Sartori, Donatella Carbonera, Leonardo Salviati, Paola Costantini
Format: Article
Language:English
Published: Nature Publishing Group 2023-12-01
Series:Cell Death and Disease
Online Access:https://doi.org/10.1038/s41419-023-06320-y

Internet

https://doi.org/10.1038/s41419-023-06320-y

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