Hypoxia rescues frataxin loss by restoring iron sulfur cluster biogenesis

Friedreich’s ataxia (FRDA) is a devastating, multisystemic disorder caused by recessive mutations in the mitochondrial protein frataxin (FXN). FXN participates in the biosynthesis of Fe-S clusters and is considered to be essential for viability. Here we report that when grown in 1% ambient O(2), FXN...

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Udgivet i:Cell
Main Authors: Ast, Tslil, Meisel, Joshua D., Patra, Shachin, Wang, Hong, Grange, Robert M. H., Kim, Sharon H., Calvo, Sarah E., Orefice, Lauren L., Nagashima, Fumiaki, Ichinose, Fumito, Zapol, Warren M., Ruvkun, Gary, Barondeau, David P., Mootha, Vamsi K.
Format: Artigo
Sprog:Inglês
Udgivet: 2019
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6911770/
https://ncbi.nlm.nih.gov/pubmed/31031004
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.cell.2019.03.045
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