...Mujan Varasteh Kia; Sharon Barone; Alicia A. McDonough; Kamyar Zahedi; Jie Xu; Manoocher Soleimani Background/Aims: Patients with cystic fibrosis (CF) are prone to the development of metabolic alkalosis; however, the pathogenesis of this life threatening derangement remains unknown. We hypothesized...

...YongTao Li; JianRong Huang; LanJuan Li; LinSheng Liu Background/Aims: Pseudomonas aeruginosa (PA) is one of the major opportunistic pathogens which can cause chronic lung infection of cystic fibrosis (CF). The formation of PA biofilm promotes CF development and restricts the antimicrobial...

...Miquéias Lopes-Pacheco; Clément Boinot; Inna Sabirzhanova; Daniele Rapino; Liudmila Cebotaru Background/Aims: Premature degradation of mutated cystic fibrosis transmembrane conductance regulator (CFTR) protein causes cystic fibrosis (CF), the commonest Mendelian disease in Caucasians. Despite...

...Barbara Kovacic; Carolin Sehl; Barbara Wilker; Markus Kamler; Katrin Anne Becker; Erich Gulbins Background : Cystic fibrosis (CF) is the most common autosomal-recessive disorder in western countries. Previous studies have demonstrated an important role of sphingolipids in the pathophysiology...

...Joachim Riethmüller; Gloria Herrmann; Ute Graepler-Mainka; Dorothea Hellwig; Hans-Eberhard Heuer; Susanne Heyder; Holger Köster; Birte Kinder; Kristina Kröger; Klaus Paul; Krystyna Poplawska; Volker O. Melichar; Christina Smaczny; Uwe Mellies Background/Aims: In cystic fibrosis (CF), chronic...

... patients. 7 06 2016 11 7 2016 Cystic fibrosis (CF) is the most common autosomal recessive disorder in western countries. The disease is caused by mutations in the cystic fibrosis transmembranous conductance regulator (CFTR) molecule [ 1,2,3 ]. The disease is characterized by chronic...

... of cystic fibrosis (CF) patients and it is believed that these infections facilitate further colonization of CF lungs with Pseudomonas aeruginosa ( P. aeruginosa ). Previous studies demonstrated a marked reduction of sphingosine in tracheal and bronchial epithelial cells in CF compared to wild type mice...

...Judith Grothe; Joachim Riethmüller; Sandra M. Tschürtz; Marco Raith; Chris J. Pynn; Dieter Stoll; Wolfgang Bernhard Background: Liver impairment, ranging from steatosis to cirrhosis, is frequent in cystic fibrosis (CF) patients and is becoming increasingly significant due to their improved life...

...Heike Grassmé; Alexander Carpinteiro; Michael J. Edwards; Erich Gulbins; Katrin Anne Becker Background: Cystic fibrosis (CF), the most common autosomal recessive disorder in Western countries, is characterized by chronic pulmonary inflammation, reduced mucociliary clearance, and increased...

... Background/Aims: Inflammation is a major and critical component of the lung pathology in the hereditary disease cystic fibrosis. The molecular mechanisms of chronic inflammation in cystic fibrosis require definition. Methods: We used several genetic mouse models to test a role of iNKT cells and ceramide...

... recent studies revealed an accumulation of ceramide in bronchial, tracheal and intestinal epithelial cells of mice and patients with cystic fibrosis (CF). Normalization of ceramide concentrations in lungs of CF mice employing the functional acid sphingomyelinase inhibitor amitriptyline also normalized...

...Filipa Mendes; Carlos M. Farinha; Verónica Felício; Paulo C. Alves; Isabel Vieira; Margarida D. Amaral Background: Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), the dysfunctional Cl - channel in Cystic Fibrosis, undergoes complex biosynthesis at the endoplasmic reticulum involving...

... with correctors and potentiators for CFTR it is important to get more information about mutant CFTR protein biochemistry. Target for novel treatment is the most afflicted organ in cystic fibrosis (CF), the lung. Methods: Lung tissue sampled from patients with CF and non-CF donors prior to lung transplantation...

...Geraldine Leier; Nadine Bangel-Ruland; Katja Sobczak; Yvonne Knieper; Wolf-Michael Weber The phosphodiesterase-5 inhibitor sildenafil is an established and approved drug to treat symptoms of a variety of human diseases. In the context of cystic fibrosis (CF), a genetic disease caused by a defective...

...Jiraporn Ousingsawat; Patthara Kongsuphol; Rainer Schreiber; Karl Kunzelmann Previous reports point out to a functional relationship of the cystic fibrosis transmembrane conductance regulator (CFTR) and Ca 2+ activated Cl - channels (CaCC). Recent findings showing that TMEM16A forms the essential...

...Nelly Weiser; Natalia Molenda; Katarina Urbanova; Martin Bähler; Uwe Pieper; Hans Oberleithner; Hermann Schillers In normal airway epithelium, the cystic fibrosis transmembrane conductance regulator (CFTR) transports Cl - ions to the apical surface of the epithelium paralleled by the flow of water...

...Beate Illek; Dachuan Lei; Horst Fischer; Dieter C. Gruenert Background/Aims: While the Cl - efflux assays are relatively straightforward, their ability to assess the efficacy of phenotypic correction in cystic fibrosis (CF) tissue or cells may be limited. Accurate assessment of therapeutic efficacy...

...Katrin Anne Becker; Heike Grassmé; Yang Zhang; Erich Gulbins Cystic fibrosis is one of the most common autosomal recessive disorders, at least in Western countries. Patients with cystic fibrosis exhibit many symptoms, the most important of which are chronic inflammation and bacterial infections...

...Monika Kloch; Michał Milewski; Ewa Nurowska; Beata Dworakowska; Garry R. Cutting; Krzysztof Dołowy The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding cassette (ABC) transporter that functions as a cAMP-activated chloride channel. The recent model of CFTR gating...

...Anabela S. Ramalho; Marzena A. Lewandowska; Carlos M. Farinha; Filipa Mendes; Juan Gonçalves; Celeste Barreto; Ann Harris; Margarida D. Amaral Background/Aims: Mutations in the CFTR gene cause Cystic Fibrosis (CF) the most common life-threatening autosomal recessive disease affecting Caucasians. We...