ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in cystic fibrosis

Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia

(2019) Katrina A. Muraglia et al.   NATURE

Brevenal, a Marine Natural Product, is Anti-Inflammatory and an Immunomodulator of Macrophage and Lung Epithelial Cells

(2019) Devon Keeler et al.   Marine Drugs

NOD-like receptors and inflammasomes: A review of their canonical and non-canonical signaling pathways

(2019) Jaye M. Platnich et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

The cumulative effect of inflammation and infection on structural lung disease in early CF

(2019) Tim Rosenow et al.   EUROPEAN RESPIRATORY JOURNAL

Muco-Obstructive Lung Diseases

(2019) Richard C. Boucher   NEW ENGLAND JOURNAL OF MEDICINE

Specific Inhibition of the NLRP3 Inflammasome as an Anti-Inflammatory Strategy in Cystic Fibrosis

(2019) Oliver J McElvaney et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Metabolic Reprograming of Cystic Fibrosis Macrophages via the IRE1α Arm of the Unfolded Protein Response Results in Exacerbated Inflammation

(2019) Samuel Lara-Reyna et al.   Frontiers in Immunology

Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still’s disease

(2018) Cem Gabay et al.   ANNALS OF THE RHEUMATIC DISEASES

Ion homeostasis and ion channels in NLRP3 inflammasome activation and regulation

(2018) Iva Hafner-Bratkovič et al.   CURRENT OPINION IN IMMUNOLOGY

The TWIK2 Potassium Efflux Channel in Macrophages Mediates NLRP3 Inflammasome-Induced Inflammation

(2018) Anke Di et al.   IMMUNITY

Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis

(2018) Samuel T. Montgomery et al.   Journal of Cystic Fibrosis

Poster Session Abstracts

(2018)   PEDIATRIC PULMONOLOGY

Chloride regulates dynamic NLRP3-dependent ASC oligomerization and inflammasome priming

(2018) Jack P. Green et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD)

(2018) George M. Solomon et al.   Jove-Journal of Visualized Experiments

Viral-Bacterial Co-infections in the Cystic Fibrosis Respiratory Tract

(2018) Megan R. Kiedrowski et al.   Frontiers in Immunology

The intracellular chloride channel proteins CLIC1 and CLIC4 induce IL-1β transcription and activate the NLRP3 inflammasome

(2017) Raquel Domingo-Fernández et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Blocking ATP-sensitive potassium channel alleviates morphine tolerance by inhibiting HSP70-TLR4-NLRP3-mediated neuroinflammation

(2017) Jie Qu et al.   Journal of Neuroinflammation

Quantification of CT bronchiectasis and its relationship to ventilation in cystic fibrosis

(2017) Tim Rosenow   THORAX

T-lymphocyte Kv1.3 channel activation triggers the NLRP3 inflammasome signaling pathway in hypertensive patients

(2017) Jian Zhu et al.   Experimental and Therapeutic Medicine

Review of autoinflammatory diseases, with a special focus on periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome

(2016) Per Wekell et al.   ACTA PAEDIATRICA

Old Dogs, New Tricks: Monogenic Autoinflammatory Disease Unleashed

(2016) Monique Stoffels et al.   Annual Review of Genomics and Human Genetics

Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies

(2016) Samuel T. Montgomery et al.   EUROPEAN RESPIRATORY JOURNAL

The burgeoning field of innate immune-mediated disease and autoinflammation

(2016) Daniel Peckham et al.   JOURNAL OF PATHOLOGY

Cystic fibrosis

(2016) J Stuart Elborn   LANCET

IL-1 receptor antagonist ameliorates inflammasome-dependent inflammation in murine and human cystic fibrosis

(2016) Rossana G. Iannitti et al.   Nature Communications

Hypoxic Epithelial Necrosis Triggers Neutrophilic Inflammation via IL-1 Receptor Signaling in Cystic Fibrosis Lung Disease

(2015) Benedikt Fritzsching et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Defective Innate Immunity and Hyperinflammation in Newborn Cystic Fibrosis Transmembrane Conductance Regulator–Knockout Ferret Lungs

(2015) Nicholas W. Keiser et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Potassium efflux fires the canon: Potassium efflux as a common trigger for canonical and noncanonical NLRP3 pathways

(2015) Jack Rivers-Auty et al.   EUROPEAN JOURNAL OF IMMUNOLOGY

Aberrant actin depolymerization triggers the pyrin inflammasome and autoinflammatory disease that is dependent on IL-18, not IL-1β

(2015) Man Lyang Kim et al.   JOURNAL OF EXPERIMENTAL MEDICINE

K+Efflux Agonists Induce NLRP3 Inflammasome Activation Independently of Ca2+Signaling

(2015) Michael A. Katsnelson et al.   JOURNAL OF IMMUNOLOGY

Respiratory epithelial cells orchestrate pulmonary innate immunity

(2015) Jeffrey A Whitsett et al.   NATURE IMMUNOLOGY

Mitochondrial Ca2+-dependent NLRP3 activation exacerbates the Pseudomonas aeruginosa-driven inflammatory response in cystic fibrosis

(2015) Alessandro Rimessi et al.   Nature Communications

A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis

(2014) M.W. Konstan et al.   Journal of Cystic Fibrosis

The adaptor ASC has extracellular and 'prionoid' activities that propagate inflammation

(2014) Bernardo S Franklin et al.   NATURE IMMUNOLOGY

K+ Efflux Is the Common Trigger of NLRP3 Inflammasome Activation by Bacterial Toxins and Particulate Matter

(2013) Raúl Muñoz-Planillo et al.   IMMUNITY

Silica induces NLRP3 inflammasome activation in human lung epithelial cells

(2013) Paul M Peeters et al.   Particle and Fibre Toxicology

The Role of Interleukin-1 and Interleukin-18 in Pro-Inflammatory and Anti-Viral Responses to Rhinovirus in Primary Bronchial Epithelial Cells

(2013) Siân C. Piper et al.   PLoS One

ENaC Inhibitors and Airway Re-hydration in Cystic Fibrosis: State of the Art

(2013) Mike Althaus   Current Molecular Pharmacology

Rhinovirus-Induced IL-1β Release from Bronchial Epithelial Cells Is Independent of Functional P2X7

(2012) Lei Shi et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Analysis of Human Bronchial Epithelial Cell Proinflammatory Response toBurkholderia cenocepaciaInfection

(2012) Devyn D. Gillette et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Inflammasome-Mediated IL-1β Production in Humans with Cystic Fibrosis

(2012) Anthony Tang et al.   PLoS One

The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease

(2011) Zhe Zhou et al.   Journal of Cystic Fibrosis

Translocated LPS Might Cause Endotoxin Tolerance in Circulating Monocytes of Cystic Fibrosis Patients

(2011) Rosa del Campo et al.   PLoS One

Sodium Overload and Water Influx Activate the NALP3 Inflammasome

(2010) Christine Schorn et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The NLRP3 inflammasome functions as a negative regulator of tumorigenesis during colitis-associated cancer

(2010) Irving C. Allen et al.   JOURNAL OF EXPERIMENTAL MEDICINE

IL-18 Production Downstream of the Nlrp3 Inflammasome Confers Protection against Colorectal Tumor Formation

(2010) M. H. Zaki et al.   JOURNAL OF IMMUNOLOGY

Potent Phagocytic Activity with Impaired Antigen Presentation Identifying Lipopolysaccharide-Tolerant Human Monocytes: Demonstration in Isolated Monocytes from Cystic Fibrosis Patients

(2009) Carlos del Fresno et al.   JOURNAL OF IMMUNOLOGY

Pyroptosis: host cell death and inflammation

(2009) Tessa Bergsbaken et al.   NATURE REVIEWS MICROBIOLOGY

An Autoinflammatory Disease with Deficiency of the Interleukin-1–Receptor Antagonist

(2009) Ivona Aksentijevich et al.   NEW ENGLAND JOURNAL OF MEDICINE

Preventive but Not Late Amiloride Therapy Reduces Morbidity and Mortality of Lung Disease in βENaC-overexpressing Mice

(2008) Zhe Zhou et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Toll-like receptors as therapeutic targets in cystic fibrosis

(2008) Catherine M Greene et al.   EXPERT OPINION ON THERAPEUTIC TARGETS

Assessment of the CFTR and ENaC association

(2008) Bakhrom K. Berdiev et al.   Molecular BioSystems

Monocytes from Cystic Fibrosis Patients Are Locked in an LPS Tolerance State: Down-Regulation of TREM-1 as Putative Underlying Mechanism

(2008) Carlos del Fresno et al.   PLoS One