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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Gepubliceerd in:Cell
Hoofdauteurs: 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.
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: 2019
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Article
Online toegang: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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