GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models

Dicarboxylate carrier-mediated glutathione transport is essential for reactive oxygen species homeostasis and normal respiration in rat brain mitochondria

(2010) Christelle K. Kamga et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Specific Contribution of Methionine and Choline in Nutritional Nonalcoholic Steatohepatitis

(2010) Francisco Caballero et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Metabolomic Profiling Reveals Biochemical Pathways and Biomarkers Associated with Pathogenesis in Cystic Fibrosis Cells

(2010) Diana R. Wetmore et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Conserved and Novel Functions forArabidopsis thalianaMIA40 in Assembly of Proteins in Mitochondria and Peroxisomes

(2010) Chris Carrie et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of Mitochondrial Glutathione Redox Status and Protein Glutathionylation by Respiratory Substrates

(2010) Jerome Garcia et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cystic Fibrosis Transmembrane Regulator Inhibitors CFTRinh-172 and GlyH-101 Target Mitochondrial Functions, Independently of Chloride Channel Inhibition

(2010) M. Kelly et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

The mitochondria-targeted anti-oxidant mitoquinone decreases liver damage in a phase II study of hepatitis C patients

(2010) Edward J. Gane et al.   LIVER INTERNATIONAL

Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

(2010) Alessandro Luciani et al.   NATURE CELL BIOLOGY

Mitochondrial Glutathione, a Key Survival Antioxidant

(2009) Montserrat Marí et al.   ANTIOXIDANTS & REDOX SIGNALING

Mitochondria, cholesterol and amyloid β peptide: a dangerous trio in Alzheimer disease

(2009) Anna Colell et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Dysfunction of mitochondria Ca2+ uptake in cystic fibrosis airway epithelial cells

(2009) Fabrice Antigny et al.   MITOCHONDRION

Peroxiredoxin 6 Fails to Limit Phospholipid Peroxidation in Lung from Cftr-Knockout Mice Subjected to Oxidative Challenge

(2009) Stéphanie Trudel et al.   PLoS One

Oxidative Stress CausesIL8Promoter Hyperacetylation in Cystic Fibrosis Airway Cell Models

(2008) Toni R. Bartling et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Hepatic mitochondrial transport of glutathione: Studies in isolated rat liver mitochondria and H4IIE rat hepatoma cells

(2008) Qing Zhong et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Complex I within Oxidatively Stressed Bovine Heart Mitochondria Is Glutathionylated on Cys-531 and Cys-704 of the 75-kDa Subunit

(2008) Thomas R. Hurd et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis

(2008) Volker Teichgräber et al.   NATURE MEDICINE

Dysfunction of Nrf-2 in CF Epithelia Leads to Excess Intracellular H2O2 and Inflammatory Cytokine Production

(2008) Junnan Chen et al.   PLoS One

Contribution of mitochondrial GSH transport to matrix GSH status and colonic epithelial cell apoptosis

(2007) Magdalena L. Circu et al.   FREE RADICAL BIOLOGY AND MEDICINE