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Article

Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency.

Nature - 1 Jan 2026

Meisel Joshua D, Joshi Pallavi R, Spelbring Amy N, Wang Hong, Wellner Sandra M, Wiesenthal Presli P, Miranda Maria, McCoy Jason G, Barondeau David P, Ruvkun Gary, Mootha Vamsi K

Abstract excerpt

Frataxin is a key component of an ancient, mitochondrial iron-sulfur cluster biosynthetic machinery, serving as an allosteric activator of the cysteine desulfurase NFS1 (refs. 1-5). Loss of frataxin levels underlies Friedreich's ataxia6, the most common inherited ataxia. Yeast, Caenorhabditis elegans and human cells can tolerate loss of frataxin when grown in 'permissive' low oxygen tensions7. Here we conducted...

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Identifiers

PMID: 41372402
DOI: 10.1038/s41586-025-09821-2
PMCID: PMC12804076

Genes

FDX2
NFS1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 3 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency.PMID 41372402Read ↗

Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarly.Semantic proximity Read ↗

Turning Saccharomyces cerevisiae into a Frataxin-Independent Organism.Semantic proximity Read ↗

Recent Advances in the Elucidation of Frataxin Biochemical Function Open Novel Perspectives for the Treatment of Friedreich’s AtaxiaSemantic proximity Read ↗

Frataxin: a protein in search for a function.Semantic proximity Read ↗

Recapitulating the frataxin activation mechanism in an engineered bacterial cysteine desulfurase supports the architectural switch modelSemantic proximity Read ↗

Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanismSemantic proximity Read ↗

Neurodegeneration in Friedreich’s Ataxia: From Defective Frataxin to Oxidative StressSemantic proximity Read ↗

Salutary Effects of Overexpression of Rsm22, an Assembly Factor for the Mitochondrial Ribosome, on Frataxin/Yfh1 Depletion Phenotypes in Saccharomyces cerevisiae.Semantic proximity Read ↗

Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster BiogenesisSemantic proximity Read ↗

Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity.Semantic proximity Read ↗

Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant.Semantic proximity Read ↗

Cells lacking pfh1, a fission yeast homolog of mammalian frataxin protein, display constitutive activation of the iron starvation response.Semantic proximity Read ↗

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