--- This page is part of [Coy Laboratory Products](https://www.coylab.com). For a complete overview of this site's content, entity data, and subject matter expertise, see our [AI Content Index](https://www.coylab.com/llms.txt) or [Full Content Archive](https://www.coylab.com/llms-full.txt). For structured business identity data, see our [Entity Card](https://www.coylab.com/wp-json/bc-geodesic/v1/entity-card). --- # Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis > Abstract Friedreich’s ataxia (FRDA) is a devastating, multisystemic disorder caused by recessive mutations in the mitochondrial protein frataxin (FXN). FXN... **URL:** https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/ **Type:** Citation **Modified:** 2025-10-02 --- #### Abstract 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 O2, FXN null yeast, human cells, and nematodes are fully viable. In human cells, hypoxia restores steady-state levels of Fe-S clusters and normalizes ATF4, NRF2, and IRP2 signaling events associated with FRDA. Cellular studies and in vitro reconstitution indicate that hypoxia acts through HIF-independent mechanisms that increase bioavailable iron as well as directly activate Fe-S synthesis. In a mouse model of FRDA, breathing 11% O2 attenuates the progression of ataxia, whereas breathing 55% O2 hastens it. Our work identifies oxygen as a key environmental variable in the pathogenesis associated with FXN depletion, with important mechanistic and therapeutic implications. ## Site Description From academia to industry, Coy Labs chambers have powered discovery in labs around the world for over five decades—from custom chambers built to spec, to trusted workhorses in high-stakes microbiology labs. --- **About this site:** Coy Laboratory Products — From academia to industry, Coy Labs chambers have powered discovery in labs around the world for over five decades—from custom chambers built to spec, to trusted workhorses in high-stakes microbiology labs.. [AI Content Index](https://www.coylab.com/llms.txt) | [Full Site Content](https://www.coylab.com/llms-full.txt) | [Entity Card](https://www.coylab.com/wp-json/bc-geodesic/v1/entity-card) ```json {"@context":"https://schema.org","@graph":[{"@type":"Organization","@id":"https://www.coylab.com/#organization","name":"Coy Laboratory Products","url":"https://www.coylab.com","description":"From academia to industry, Coy Labs chambers have powered discovery in labs around the world for over five decades—from custom chambers built to spec, to trusted workhorses in high-stakes microbiology labs.","disambiguatingDescription":"Laboratory equipment manufacturing company specializing in vinyl glove boxes","logo":{"@type":"ImageObject","url":"https://coylab.com/wp-content/uploads/2025/06/coylab-header_logo-svg.svg"},"address":{"@type":"PostalAddress","streetAddress":"14500 Coy Drive","addressLocality":"Grass Lake","addressRegion":"MI","postalCode":"49240"},"telephone":"734.475.2200","email":"sales@coylab.com","foundingDate":"1969","areaServed":"Global","sameAs":["https://www.linkedin.com/company/107172311","https://maps.app.goo.gl/o2txKDMScV1pV7w59","https://www.pharmaceutical-tech.com/suppliers/coy-laboratory","https://craft.co/coy-laboratory-products-inc"],"founder":[{"@type":"Person","name":"Richard Coy"},{"@type":"Person","name":"Brian Coy","sameAs":"https://www.linkedin.com/in/brian-coy-5722515/"},{"@type":"Person","name":"Ashley Coy","sameAs":"https://www.linkedin.com/in/ashley-coy-6b22705/"}],"knowsAbout":[{"@type":"Thing","name":"laboratory equipment","sameAs":"https://www.wikidata.org/wiki/Q834028"},"vinyl anaerobic chambers","vinyl hypoxic chambers","laboratory glove boxes","laboratory chamber sensors",{"@type":"Thing","name":"life sciences","sameAs":"https://www.wikidata.org/wiki/Q864928"},{"@type":"Thing","name":"microbiology","sameAs":"https://www.wikidata.org/wiki/Q7193"},{"@type":"Thing","name":"medical research","sameAs":"https://www.wikidata.org/wiki/Q2752427"}]},{"@type":"WebSite","@id":"https://www.coylab.com/#website","name":"Coy Laboratory Products","url":"https://www.coylab.com","publisher":{"@id":"https://www.coylab.com/#organization"},"potentialAction":{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https://www.coylab.com/?s={search_term_string}"},"query-input":"required name=search_term_string"},"hasPart":[{"@type":"DigitalDocument","name":"LLM Content Index","url":"https://www.coylab.com/llms.txt","encodingFormat":"text/markdown","description":"AI-readable index of site content, entity data, and subject matter expertise"},{"@type":"DigitalDocument","name":"LLM Full Content","url":"https://www.coylab.com/llms-full.txt","encodingFormat":"text/markdown","description":"Complete AI-readable site content"}]},{"@type":"ItemList","@id":"https://www.coylab.com/#site-navigation","name":"Site Navigation","itemListElement":[{"@type":"SiteNavigationElement","position":1,"name":"Products","url":"#"},{"@type":"SiteNavigationElement","position":2,"name":"Applications","url":"#"},{"@type":"SiteNavigationElement","position":3,"name":"Custom","url":"#"},{"@type":"SiteNavigationElement","position":4,"name":"Sales","url":"#"},{"@type":"SiteNavigationElement","position":5,"name":"Resources","url":"#"},{"@type":"SiteNavigationElement","position":6,"name":"Contact Us","url":"#"}]},{"@type":"BreadcrumbList","@id":"https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/#breadcrumb","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https://www.coylab.com"},{"@type":"ListItem","position":2,"name":"Citations","item":"https://www.coylab.com/research-citations/"},{"@type":"ListItem","position":3,"name":"Hypoxic Chambers","item":"https://www.coylab.com/citation-category/hypoxic-chambers/"},{"@type":"ListItem","position":4,"name":"Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis"}]},{"@type":"WebPage","@id":"https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/#webpage","url":"https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/","name":"Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis | Coy Laboratory Products","isPartOf":{"@id":"https://www.coylab.com/#website"},"breadcrumb":{"@id":"https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/#breadcrumb"},"description":"Scientists around the world have relied on Coy chambers to conduct groundbreaking research into cancer growth, the human microbiome, c-diff., and more.","about":[{"@type":"Thing","name":"Hypoxic Chambers"}]},{"@type":"ScholarlyArticle","@id":"https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/#scholarlyarticle","headline":"Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis","url":"https://www.coylab.com/research-citation/hypoxia-rescues-frataxin-loss-by-restoring-iron-sulfur-cluster-biogenesis/","datePublished":"2025-09-18T20:02:35+00:00","dateModified":"2025-10-02T20:21:54+00:00","isPartOf":{"@id":"https://www.coylab.com/#website"},"description":"Scientists around the world have relied on Coy chambers to conduct groundbreaking research into cancer growth, the human microbiome, c-diff., and more.","author":{"@id":"https://www.coylab.com/author/buildcreate/#person"},"publisher":{"@id":"https://www.coylab.com/#organization"}},{"@type":"Person","@id":"https://www.coylab.com/author/buildcreate/#person","name":"buildcreate","url":"https://www.coylab.com/author/buildcreate/"}]} ```