GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models

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 imba...

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Veröffentlicht in:	Human molecular genetics 2011-07, Vol.20 (14), p.2745-2759
Hauptverfasser:	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
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Biological and medical sciences Cell Line Cystic Fibrosis - drug therapy Cystic Fibrosis - metabolism Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Dicarboxylic Acid Transporters - genetics Dicarboxylic Acid Transporters - metabolism Electron Transport Complex I - genetics Electron Transport Complex I - metabolism Errors of metabolism Fundamental and applied biological sciences. Psychology Genetics of eukaryotes. Biological and molecular evolution Glutathione - analogs & derivatives Glutathione - pharmacology Interleukin-8 - metabolism Male Medical sciences Membrane Potential, Mitochondrial - drug effects Membrane Transport Proteins - genetics Membrane Transport Proteins - metabolism Metabolic diseases Mice Mice, Inbred CFTR Mice, Knockout Miscellaneous hereditary metabolic disorders Mitochondria - genetics Mitochondria - metabolism Mitochondria - pathology Molecular and cellular biology Mutation Radiation-Protective Agents - pharmacology Recovery of Function - drug effects
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