Human epididymis protein 4 (HE4) protects against cystic pulmonary fibrosis associated-inflammation through inhibition of NF-κB and MAPK singnaling

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

Volker Teichgräber, Martina Ulrich, Nicole Endlich et al. · Nature Medicine · 2008 · 536 citations

Life Sciences, Cell Biology, Pathobiology +17

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

Alessandro Luciani, Valeria Rachela Villella, Speranza Esposito et al. · Nature Cell Biology · 2010 · 460 citations

Life Sciences, Cell Biology, Molecular Genetics +17

Evidence for airway surface dehydration as the initiating event in CF airway disease

Richard C. Boucher · Journal of Internal Medicine · 2007 · 351 citations · Full text

Life Sciences, Microbiology, Respiratory Therapy +17

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial

Eitan Kerem, Michael W. Konstan, K. De Boeck et al. · The Lancet Respiratory Medicine · 2014 · 335 citations · Full text

Medicine, Molecular Medicine, Pharmacotherapy +9

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

Viral S. Shah, David K. Meyerholz, Xiao Xiao Tang et al. · Science · 2016 · 292 citations · Full text

Life Sciences, Pharmacology, Physiology +17