Rethinking cystic fibrosis pathology: the critical role of abnormal reduced glutathione (GSH) transport caused by CFTR mutation

VM Hudson - Free radical biology and medicine, 2001 - Elsevier
Though the cause of cystic fibrosis (CF) pathology is understood to be the mutation of the
CFTR protein, it has been difficult to trace the exact mechanisms by which the pathology …

New insights into the pathogenesis of cystic fibrosis: pivotal role of glutathione system dysfunction and implications for therapy

VM Hudson - Treatments in respiratory medicine, 2004 - Springer
The cystic fibrosis transmembrane regulator (CFTR) should no longer be viewed primarily as
a 'chloride channel'but recognized as a channel that also controls the efflux of other …

[HTML][HTML] Improvement in clinical markers in CF patients using a reduced glutathione regimen: an uncontrolled, observational study

A Visca, CT Bishop, SC Hilton, VM Hudson - Journal of Cystic Fibrosis, 2008 - Elsevier
CFTR mutation, which causes cystic fibrosis (CF), has also recently been identified as
causing glutathione system dysfunction and systemic deficiency of reduced glutathione …

A new model of cystic fibrosis pathology: lack of transport of glutathione and its thiocyanate conjugates

M Childers, G Eckel, A Himmel, J Caldwell - Medical hypotheses, 2007 - Elsevier
Many of the symptoms of cystic fibrosis are not explained by the current disease
mechanisms. Therefore, the authors conducted an extensive literature review and present a …

GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models

M Kelly-Aubert, S Trudel, J Fritsch… - Human molecular …, 2011 - academic.oup.com
Cystic fibrosis (CF), a multisystem disease caused by CFTR (cystic fibrosis transmembrane
conductance regulator) gene mutations, is associated with an abnormal inflammatory …

Systemic deficiency of glutathione in cystic fibrosis

JH Roum, R Buhl, NG McElvaney… - Journal of applied …, 1993 - journals.physiology.org
Cystic fibrosis (CF), a disorder characterized by mutations of the CF transmembrane
regulator gene, is characterized in the lung by chronic inflammation, leading to progressive …

[HTML][HTML] CFTR directly mediates nucleotide-regulated glutathione flux

I Kogan, M Ramjeesingh, C Li, JF Kidd, Y Wang… - The EMBO …, 2003 - embopress.org
Studies have shown that expression of cystic fibrosis transmembrane conductance regulator
(CFTR) is associated with enhanced glutathione (GSH) efflux from airway epithelial cells …

Cystic fibrosis and oxidative stress: the role of CFTR

E Moliteo, M Sciacca, A Palmeri, M Papale, S Manti… - Molecules, 2022 - mdpi.com
There is substantial evidence in the literature that patients with cystic fibrosis (CF) have
higher oxidative stress than patients with other diseases or healthy subjects. This results in …

Abnormal glutathione transport in cystic fibrosis airway epithelia

L Gao, KJ Kim, JR Yankaskas… - American Journal of …, 1999 - journals.physiology.org
Glutathione (GSH) is a potentially important component of antioxidant defense in the
epithelial lung lining fluid. Cystic fibrosis (CF) patients have chronic inflammation in which …

Cystic fibrosis: pathophysiology of lung disease

C Bergeron, AM Cantin - Seminars in respiratory and critical …, 2019 - thieme-connect.com
Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …