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Article

GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models.

Human molecular genetics - 15 Jul 2011

Kelly-Aubert Mairead, Trudel Stéphanie, Fritsch Janine, Nguyen-Khoa Thao, Baudouin-Legros Maryvonne, Moriceau Sandra, Jeanson Ludovic, Djouadi Fatima, Matar Corine, Conti Marc, Ollero Mario, Brouillard Franck, Edelman Aleksander

Abstract excerpt

Cystic fibrosis (CF), a multisystem disease caused by CFTR (cystic fibrosis transmembrane conductance regulator) gene mutations, is associated with an abnormal inflammatory response and compromised redox homeostasis in the airways. Recent evidence suggests that dysfunctional CFTR leads to redox imbalance and to mitochondrial reduced glutathione (mtGSH) depletion in CF models. This study was designed to...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Cystic Fibrosis Transmembrane Conductance Regulator

Share to Electron Transport Complex I

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Share to Interleukin-8

Share to Membrane Potential, Mitochondrial

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Source

Identifiers

PMID: 21518732
DOI: 10.1093/hmg/ddr173

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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