Mucosal Immunity in Cystic Fibrosis

Cytokine signaling pathway in cystic fibrosis: expression of SOCS and STATs genes in different clinical phenotypes of the disease

(2021) Swati Sagwal et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis

(2020) Heledd H Jarosz-Griffiths et al.   eLife

CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine

(2020) Miquéias Lopes-Pacheco   Frontiers in Pharmacology

Immunomodulation in Cystic Fibrosis: Why and How?

(2020) Vincent D. Giacalone et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Intrinsic alterations in peripheral neutrophils from cystic fibrosis newborn piglets

(2020) Déborah Bréa et al.   Journal of Cystic Fibrosis

Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil Activity

(2020) Daniel R. Laucirica et al.   Frontiers in Immunology

ILC2 Lung-Homing in Cystic Fibrosis Patients: Functional Involvement of CCR6 and Impact on Respiratory Failure

(2020) Anja Schulz-Kuhnt et al.   Frontiers in Immunology

Targeting IgG Autoantibodies for Improved Cytotoxicity of Bactericidal Permeability Increasing Protein in Cystic Fibrosis

(2020) Karen McQuillan et al.   Frontiers in Pharmacology

CFTR Modulator Therapy Enhances Peripheral Blood Monocyte Contributions to Immune Responses in People With Cystic Fibrosis

(2020) Katherine B. Hisert et al.   Frontiers in Pharmacology

Ivacaftor Modifies Cystic Fibrosis Neutrophil Phenotype in Subjects with R117H Residual Function CFTR Mutations

(2020) Gareth R. Hardisty et al.   EUROPEAN RESPIRATORY JOURNAL

Airway Inflammation and Host Responses in the Era of CFTR Modulators

(2020) Karen Keown et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Lung immunoglobulin A immunity dysregulation in cystic fibrosis

(2020) Amandine M. Collin et al.   EBioMedicine

CFTR Protects against Mycobacterium abscessus Infection by Fine-Tuning Host Oxidative Defenses

(2019) Audrey Bernut et al.   Cell Reports

Group 2 Innate Lymphoid Cells in Human Respiratory Disorders

(2019) Esmee K. van der Ploeg et al.   Journal of Innate Immunity

Pendrin Mediates Bicarbonate Secretion and Enhances CFTR Function in Airway Surface Epithelia

(2019) Dusik Kim et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Transcriptomic responses to Ivacaftor and prediction of Ivacaftor clinical responsiveness

(2019) Tao Sun et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Clinically approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia

(2019) Dana C. Borcherding et al.   JOURNAL OF CLINICAL INVESTIGATION

IL-1β, IL-23, and TGF-β drive plasticity of human ILC2s towards IL-17-producing ILCs in nasal inflammation

(2019) Korneliusz Golebski et al.   Nature Communications

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

Cystic Fibrosis Lung Disease and Immunometabolism: Targeting the NLRP3 Inflammasome

(2019) André M. Cantin   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis

(2019) Benjamin T. Kopp et al.   Journal of Cystic Fibrosis

Human cystic fibrosis monocyte derived macrophages display no defect in acidification of phagolysosomes when measured by optical nanosensors

(2019) Sheonagh M. Law et al.   Journal of Cystic Fibrosis

ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel

(2019) Yu-Sheng Wu et al.   MOLECULAR PHARMACOLOGY

The role of vitamin D in increasing circulating T regulatory cell numbers and modulating T regulatory cell phenotypes in patients with inflammatory disease or in healthy volunteers: A systematic review

(2019) Sheila A. Fisher et al.   PLoS One

Mycobacterium abscessus, an Emerging and Worrisome Pathogen among Cystic Fibrosis Patients

(2019) Degiacomi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

(2019) Peter G. Middleton et al.   NEW ENGLAND JOURNAL OF MEDICINE

Cystic Fibrosis Disease Modifiers: Complex Genetics Defines the Phenotypic Diversity in a Monogenic Disease

(2018) Wanda K. O'Neal et al.   Annual Review of Genomics and Human Genetics

Mycobacterium abscessus Smooth and Rough Morphotypes Form Antimicrobial-Tolerant Biofilm Phenotypes but Are Killed by Acetic Acid

(2018) Gillian Clary et al.   ANTIMICROBIAL AGENTS AND CHEMOTHERAPY

Antimicrobial Activity of Ibuprofen against Cystic Fibrosis-Associated Gram-Negative Pathogens

(2018) Parth N. Shah et al.   ANTIMICROBIAL AGENTS AND CHEMOTHERAPY

Severely Impaired Control of Bacterial Infections in a Patient With Cystic Fibrosis Defective in Mucosal-Associated Invariant T Cells

(2018) Terezia Pincikova et al.   CHEST

Increased platelet activation occurs in cystic fibrosis patients and correlates to clinical status

(2018) Ulrika Lindberg et al.   THROMBOSIS RESEARCH

Anomalies in T Cell Function Are Associated With Individuals at Risk of Mycobacterium abscessus Complex Infection

(2018) Viviana P. Lutzky et al.   Frontiers in Immunology

Specialized Pro-Resolving Lipid Mediators in Cystic Fibrosis

(2018) Réginald Philippe et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

TNFα −857 C/T and TNFR2 +587 T/G polymorphisms are associated with cystic fibrosis in Iranian patients

(2018) Maryam Hassanzad et al.   European Journal of Medical Genetics

Rapid therapeutic advances in CFTR modulator science

(2018) John P. Clancy   PEDIATRIC PULMONOLOGY

Pulmonary function patterns and their association with genotype and phenotype in adult cystic fibrosis patients

(2018) Dimitri Stylemans et al.   ACTA CLINICA BELGICA

Vitamin D for the Immune System in Cystic Fibrosis (DISC): a double-blind, multicenter, randomized, placebo-controlled clinical trial

(2018) Vin Tangpricha et al.   AMERICAN JOURNAL OF CLINICAL NUTRITION

Bacteria-driven peribronchial lymphoid neogenesis in bronchiectasis and cystic fibrosis

(2017) Justine Frija-Masson et al.   EUROPEAN RESPIRATORY JOURNAL

Impaired innate immune cells in cystic fibrosis: Is it really a surprise?

(2017) Tracey Bonfield et al.   Journal of Cystic Fibrosis

Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth

(2017) Lily Paemka et al.   Journal of Cystic Fibrosis

CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis

(2017) Abdullah A. Tarique et al.   Journal of Cystic Fibrosis

Neutrophil extracellular traps and the dysfunctional innate immune response of cystic fibrosis lung disease: a review

(2017) Sheonagh M. Law et al.   Journal of Inflammation-London

A mast cell-ILC2-Th9 pathway promotes lung inflammation in cystic fibrosis

(2017) Silvia Moretti et al.   Nature Communications

DNA methylation at modifier genes of lung disease severity is altered in cystic fibrosis

(2017) Milena Magalhães et al.   Clinical Epigenetics

Elevated IgG4 serum levels in patients with cystic fibrosis

(2017) Axelle Clerc et al.   PLoS One

Role of CFTR in epithelial physiology

(2016) Vinciane Saint-Criq et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Innate and Adaptive Immunity in Cystic Fibrosis

(2016) Emanuela M. Bruscia et al.   CLINICS IN CHEST MEDICINE

Clinical impact of vitamin D treatment in cystic fibrosis: a pilot randomized, controlled trial

(2016) T Pincikova et al.   EUROPEAN JOURNAL OF CLINICAL NUTRITION

HLA-G expression and regulation during Pseudomonas aeruginosa infection in cystic fibrosis patients

(2016) Roberta Rizzo et al.   Future Microbiology

Current Concepts and Controversies in Innate Immunity of Cystic Fibrosis Lung Disease

(2016) Anjali Ralhan et al.   Journal of Innate Immunity

Cystic Fibrosis Lung Immunity: The Role of the Macrophage

(2016) Emanuela M. Bruscia et al.   Journal of Innate Immunity

Genotype–phenotype correlation and functional studies in patients with cystic fibrosis bearing CFTR complex alleles

(2016) Vito Terlizzi et al.   JOURNAL OF MEDICAL GENETICS

Cystic fibrosis

(2016) J Stuart Elborn   LANCET

Molecular Genetics of Cystic Fibrosis Transmembrane Conductance Regulator

(2016) Patrick R. Sosnay et al.   PEDIATRIC CLINICS OF NORTH AMERICA

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

(2016) V. S. Shah et al.   SCIENCE

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

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

Pseudomonas aeruginosa Airway Infection Recruits and Modulates Neutrophilic Myeloid-Derived Suppressor Cells

(2016) Hasan H. Öz et al.   Frontiers in Cellular and Infection Microbiology

Gene Expression in Transformed Lymphocytes Reveals Variation in Endomembrane and HLA Pathways Modifying Cystic Fibrosis Pulmonary Phenotypes

(2015) Wanda K. O’Neal et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Junctional abnormalities in human airway epithelial cells expressing F508del CFTR

(2015) Samuel A. Molina et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Regulatory T Cells in Cystic Fibrosis Lung Disease. More Answers, More Questions

(2015) John K. McGuire   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Regulatory T-Cell Impairment in Cystic Fibrosis Patients with ChronicPseudomonasInfection

(2015) Andreas Hector 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

Epigenetics in Cystic Fibrosis: Epigenetic Targeting of a Genetic Disease

(2015) Nualpun Sirinupong et al.   CURRENT DRUG TARGETS

Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis

(2015) William P. Sexauer et al.   Journal of Cystic Fibrosis

Increasing nontuberculous mycobacteria infection in cystic fibrosis

(2015) Ophir Bar-On et al.   Journal of Cystic Fibrosis

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015)   NEW ENGLAND JOURNAL OF MEDICINE

Inflammation and its genesis in cystic fibrosis

(2015) David P. Nichols et al.   PEDIATRIC PULMONOLOGY

High Peripheral Blood Th17 Percent Associated with Poor Lung Function in Cystic Fibrosis

(2015) Emily M. Mulcahy et al.   PLoS One

Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis

(2015) Harriet Corvol et al.   Nature Communications

Exome Sequencing of Phenotypic Extremes Identifies CAV2 and TMC6 as Interacting Modifiers of Chronic Pseudomonas aeruginosa Infection in Cystic Fibrosis

(2015) Mary J. Emond et al.   PLoS Genetics

Invariant Natural Killer T (iNKT) Cells Prevent Autoimmunity, but Induce Pulmonary Inflammation in Cystic Fibrosis

(2014) Nanna Siegmann et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

CD4+CD25highFOXP3+Regulatory T Cells Correlate with FEV1in North Indian Children with Cystic Fibrosis

(2014) Nidhi Anil et al.   IMMUNOLOGICAL INVESTIGATIONS

Lung disease modifier genes in cystic fibrosis

(2014) Loic Guillot et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Beyond the Genome: Epigenetic Mechanisms in Lung Remodeling

(2014) James S. Hagood   PHYSIOLOGY

Neutrophil-Mediated Phagocytic Host Defense Defect in Myeloid Cftr-Inactivated Mice

(2014) Hang Pong Ng et al.   PLoS One

Reduced Mucosal Associated Invariant T-Cells Are Associated with Increased Disease Severity and Pseudomonas aeruginosa Infection in Cystic Fibrosis

(2014) Daniel J. Smith et al.   PLoS One

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

(2014) M. J. Hoegger et al.   SCIENCE

A Th17- and Th2-skewed Cytokine Profile in Cystic Fibrosis Lungs Represents a Potential Risk Factor forPseudomonas aeruginosaInfection

(2013) Kerstin Tiringer et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Characterization of Rough and Smooth Morphotypes of Mycobacterium abscessus Isolates from Clinical Specimens

(2013) K. Ruger et al.   JOURNAL OF CLINICAL MICROBIOLOGY

Mucus Removal Is Impaired in Children with Cystic Fibrosis Who Have Been Infected by Pseudomonas aeruginosa

(2013) Beth L. Laube et al.   JOURNAL OF PEDIATRICS

Ivacaftor in a G551D Homozygote with Cystic Fibrosis

(2013) Michael J. Harrison et al.   NEW ENGLAND JOURNAL OF MEDICINE

Impaired Functions of Macrophage from Cystic Fibrosis Patients: CD11b, TLR-5 Decrease and sCD14, Inflammatory Cytokines Increase

(2013) Karin Simonin-Le Jeune et al.   PLoS One

Intrinsic predisposition of naïve cystic fibrosis T cells to differentiate towards a Th17 phenotype

(2013) Rahul Kushwah et al.   RESPIRATORY RESEARCH

Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications

(2013) Malena Cohen-Cymberknoh et al.   THORAX

Antibiotic and Anti-Inflammatory Therapies for Cystic Fibrosis

(2013) J. F. Chmiel et al.   Cold Spring Harbor Perspectives in Medicine

THE ROLE OF LIPOXIN A4 IN CYSTIC FIBROSIS LUNG DISEASE

(2013) Valérie Urbach et al.   Computational and Structural Biotechnology Journal

Immune Responses in Cystic Fibrosis

(2012) Dmitry Ratner et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Phagocytosis and cytokine response to rough and smooth colony variants ofMycobacterium abscessusby human peripheral blood mononuclear cells

(2012) Bodil Jönsson et al.   APMIS

Patients with cystic fibrosis have inducible IL-17+IL-22+ memory cells in lung draining lymph nodes

(2012) Yvonne R. Chan et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

An Update on the Screening, Diagnosis, Management, and Treatment of Vitamin D Deficiency in Individuals with Cystic Fibrosis: Evidence-Based Recommendations from the Cystic Fibrosis Foundation

(2012) V. Tangpricha et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Innate immunity in cystic fibrosis lung disease

(2012) D. Hartl et al.   Journal of Cystic Fibrosis

Absence of the cystic fibrosis transmembrane regulator (Cftr ) from myeloid-derived cells slows resolution of inflammation and infection

(2012) T. L. Bonfield et al.   JOURNAL OF LEUKOCYTE BIOLOGY

HLA-DRB1 and HLA-DQB1 genes on susceptibility to and protection from allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

(2012) Manuel Muro et al.   MICROBIOLOGY AND IMMUNOLOGY

Proinflammatory cytokine secretion is suppressed by TMEM16A or CFTR channel activity in human cystic fibrosis bronchial epithelia

(2012) Guido Veit et al.   MOLECULAR BIOLOGY OF THE CELL

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

(2012) Alejandro A. Pezzulo et al.   NATURE

AGER -429T/C Is Associated with an Increased Lung Disease Severity in Cystic Fibrosis

(2012) Julie Beucher et al.   PLoS One

The role of matrix metalloproteinases in cystic fibrosis lung disease

(2011) A. Gaggar et al.   EUROPEAN RESPIRATORY JOURNAL

Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions

(2011) Daniel Lorentzen et al.   FREE RADICAL BIOLOGY AND MEDICINE

Cystic fibrosis modifier genes related to Pseudomonas aeruginosa infection

(2011) J E Park et al.   GENES AND IMMUNITY

Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice

(2011) Xiao Su et al.   INFLAMMATION RESEARCH

Change in IgG and evolution of lung function in children with cystic fibrosis

(2011) M. Proesmans et al.   Journal of Cystic Fibrosis

An overview of international literature from cystic fibrosis registries. Part 3. Disease incidence, genotype/phenotype correlation, microbiology, pregnancy, clinical complications, lung transplantation, and miscellanea

(2011) Donatello Salvatore et al.   Journal of Cystic Fibrosis

Association between genotype and pulmonary phenotype in cystic fibrosis patients with severe mutations

(2011) A. Geborek et al.   Journal of Cystic Fibrosis

Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2

(2011) Fred A Wright et al.   NATURE GENETICS

The development of inducible bronchus-associated lymphoid tissue depends on IL-17

(2011) Javier Rangel-Moreno et al.   NATURE IMMUNOLOGY

Cystic fibrosis, atopy, asthma and ABPA

(2010) J. Antunes et al.   ALLERGOLOGIA ET IMMUNOPATHOLOGIA

A polymorphism in the 5’ UTR of the DEFB1 gene is associated with the lung phenotype in F508del homozygous Italian cystic fibrosis patients

(2010) Sergio Crovella et al.   CLINICAL CHEMISTRY AND LABORATORY MEDICINE

Inverse relation between vitamin D and serum total immunoglobulin G in the Scandinavian Cystic Fibrosis Nutritional Study

(2010) T Pincikova et al.   EUROPEAN JOURNAL OF CLINICAL NUTRITION

Cystic fibrosis transmembrane conductance regulator (CFTR) expression in human platelets: impact on mediators and mechanisms of the inflammatory response

(2010) Domenico Mattoscio et al.   FASEB JOURNAL

Epigenetics and gene expression

(2010) E R Gibney et al.   HEREDITY

Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis

(2010) Xingshen Sun et al.   JOURNAL OF CLINICAL INVESTIGATION

Airway remodelling and its relationship to inflammation in cystic fibrosis

(2010) N. Regamey et al.   THORAX

T-helper Type 2–driven Inflammation Defines Major Subphenotypes of Asthma

(2009) Prescott G. Woodruff et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Mucins, Mucus, and Sputum

(2009) Judith A. Voynow et al.   CHEST

Disease-causing Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator Determine the Functional Responses of Alveolar Macrophages

(2009) Ludmila V. Deriy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease

(2009) YuanYuan Gu et al.   NATURE

IL1Bpolymorphisms modulate cystic fibrosis lung disease

(2009) Hara Levy et al.   PEDIATRIC PULMONOLOGY

Histo-Blood Group Gene Polymorphisms as Potential Genetic Modifiers of Infection and Cystic Fibrosis Lung Disease Severity

(2009) Jennifer L. Taylor-Cousar et al.   PLoS One

The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases

(2009) Y. Xu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Interleukin-17A induces bicarbonate secretion in normal human bronchial epithelial cells

(2008) James L. Kreindler et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Airway Inflammation in Cystic Fibrosis

(2008) Arnon Elizur et al.   CHEST

Update on gene modifiers in cystic fibrosis

(2008) Joseph M Collaco et al.   CURRENT OPINION IN PULMONARY MEDICINE

Cystic fibrosis patients' B-lymphocyte response is resistant to the in vitro enhancing effect of corticosteroids

(2008) D. EMILIE et al.   EUROPEAN JOURNAL OF CLINICAL INVESTIGATION

Modulation of cystic fibrosis lung disease by variants in interleukin-8

(2008) A D Hillian et al.   GENES AND IMMUNITY

Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders

(2008) Samuel M Moskowitz et al.   GENETICS IN MEDICINE

Complex two-gene modulation of lung disease severity in children with cystic fibrosis

(2008) Ruslan Dorfman et al.   JOURNAL OF CLINICAL INVESTIGATION

Immunological mechanisms behind the cystic fibrosis-ABPA link

(2008) Dominik Hartl   MEDICAL MYCOLOGY