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Beyond readthrough: ataluren restores mitochondrial function and reduces oxidative stress in FANCA-mutated cells via mTOR-DRP1 modulation.

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Title: Beyond readthrough: ataluren restores mitochondrial function and reduces oxidative stress in FANCA-mutated cells via mTOR-DRP1 modulation.
Authors: Balbi M; Department of Experimental Medicine, University of Genoa, Genoa, Italy., Guidi E; Department of Experimental Medicine, University of Genoa, Genoa, Italy., Hristodor AM; Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Corsolini F; Unit of Hematology, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Cossu V; Department of Experimental Medicine, University of Genoa, Genoa, Italy. vanessa.cossu@edu.unige.it.; IRCCS Ospedale Policlinico San Martino, Genoa, Italy. vanessa.cossu@edu.unige.it., Bottega R; Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy., Serra M; Department of Experimental Medicine, University of Genoa, Genoa, Italy., Pestarino S; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, and Maternal and Child Health, University of Genoa, Genoa, Italy.; Hematopoietic Stem Cell Transplantation Unit, Department of Pediatric Hematology-Oncology, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Bartolucci M; Clinical Proteomics and Metabolomics Core Facility, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Cipolli M; Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Regis S; UOSD Laboratorio di Terapie Cellulari, IRCCS Istituto Giannina Gaslini, Genova, Italy., Bezzerri V; Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.; Department of Life Sciences, Health and Health Professions, Link Campus University, Rome, Italy., Cappelli E; Unit of Hematology, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Ravera S; Department of Experimental Medicine, University of Genoa, Genoa, Italy.; IRCCS Ospedale Policlinico San Martino, Genoa, Italy.
Source: Cell death discovery [Cell Death Discov] 2026 Feb 28; Vol. 12 (1). Date of Electronic Publication: 2026 Feb 28.
Publication Type: Journal Article
Journal Info: Publisher: Nature Publishing Group Country of Publication: United States NLM ID: 101665035 Publication Model: Electronic Cited Medium: Print ISSN: 2058-7716 (Print) Linking ISSN: 20587716 NLM ISO Abbreviation: Cell Death Discov Subsets: PubMed not MEDLINE
Database: MEDLINE Ultimate
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  Data: Beyond readthrough: ataluren restores mitochondrial function and reduces oxidative stress in FANCA-mutated cells via mTOR-DRP1 modulation.
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  Data: <searchLink fieldCode="AU" term="%22Balbi+M%22">Balbi M</searchLink>; Department of Experimental Medicine, University of Genoa, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Guidi+E%22">Guidi E</searchLink>; Department of Experimental Medicine, University of Genoa, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Hristodor+AM%22">Hristodor AM</searchLink>; Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.<br /><searchLink fieldCode="AU" term="%22Corsolini+F%22">Corsolini F</searchLink>; Unit of Hematology, IRCCS Istituto Giannina Gaslini, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Cossu+V%22">Cossu V</searchLink>; Department of Experimental Medicine, University of Genoa, Genoa, Italy. vanessa.cossu@edu.unige.it.; IRCCS Ospedale Policlinico San Martino, Genoa, Italy. vanessa.cossu@edu.unige.it.<br /><searchLink fieldCode="AU" term="%22Bottega+R%22">Bottega R</searchLink>; Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy.<br /><searchLink fieldCode="AU" term="%22Serra+M%22">Serra M</searchLink>; Department of Experimental Medicine, University of Genoa, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Pestarino+S%22">Pestarino S</searchLink>; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, and Maternal and Child Health, University of Genoa, Genoa, Italy.; Hematopoietic Stem Cell Transplantation Unit, Department of Pediatric Hematology-Oncology, IRCCS Istituto Giannina Gaslini, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Bartolucci+M%22">Bartolucci M</searchLink>; Clinical Proteomics and Metabolomics Core Facility, IRCCS Istituto Giannina Gaslini, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Cipolli+M%22">Cipolli M</searchLink>; Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.<br /><searchLink fieldCode="AU" term="%22Regis+S%22">Regis S</searchLink>; UOSD Laboratorio di Terapie Cellulari, IRCCS Istituto Giannina Gaslini, Genova, Italy.<br /><searchLink fieldCode="AU" term="%22Bezzerri+V%22">Bezzerri V</searchLink>; Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.; Department of Life Sciences, Health and Health Professions, Link Campus University, Rome, Italy.<br /><searchLink fieldCode="AU" term="%22Cappelli+E%22">Cappelli E</searchLink>; Unit of Hematology, IRCCS Istituto Giannina Gaslini, Genoa, Italy.<br /><searchLink fieldCode="AU" term="%22Ravera+S%22">Ravera S</searchLink>; Department of Experimental Medicine, University of Genoa, Genoa, Italy.; IRCCS Ospedale Policlinico San Martino, Genoa, Italy.
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  Data: <i>Publisher: </i><searchLink fieldCode="PB" term="%22Nature+Publishing+Group%22">Nature Publishing Group </searchLink><i>Country of Publication: </i>United States <i>NLM ID: </i>101665035 <i>Publication Model: </i>Electronic <i>Cited Medium: </i>Print <i>ISSN: </i>2058-7716 (Print) <i>Linking ISSN: </i><searchLink fieldCode="IS" term="%2220587716%22">20587716 </searchLink><i>NLM ISO Abbreviation: </i>Cell Death Discov <i>Subsets: </i>PubMed not MEDLINE
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