- Home
- Publications
- Publication Search
- Publication Details
Title
Cystic fibrosis: a mucosal immunodeficiency syndrome
Authors
Keywords
-
Journal
NATURE MEDICINE
Volume 18, Issue 4, Pages 509-519
Publisher
Springer Nature
Online
2012-04-06
DOI
10.1038/nm.2715
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- High Mobility Group Protein-1 Inhibits Phagocytosis of Apoptotic Neutrophils through Binding to Phosphatidylserine
- (2014) G. Liu et al. JOURNAL OF IMMUNOLOGY
- The Pseudomonas Toxin Pyocyanin Inhibits the Dual Oxidase-Based Antimicrobial System as It Imposes Oxidative Stress on Airway Epithelial Cells
- (2014) B. Rada et al. JOURNAL OF IMMUNOLOGY
- Mucous solids and liquid secretion by airways: studies with normal pig, cystic fibrosis human, and non-cystic fibrosis human bronchi
- (2011) Chelsea J. Martens et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Infection, Inflammation, and Lung Function Decline in Infants with Cystic Fibrosis
- (2011) Naveen Pillarisetti et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Pseudomonas aeruginosaPopulation Diversity and Turnover in Cystic Fibrosis Chronic Infections
- (2011) Eilidh Mowat et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- The Th17 Pathway in Cystic Fibrosis Lung Disease
- (2011) Hui-Leng Tan et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- IL-17 in Cystic Fibrosis: More Than Just Th17 Cells
- (2011) Patricia J. Dubin et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Nebulized Hypertonic Saline Decreases IL-8 in Sputum of Patients with Cystic Fibrosis
- (2011) Emer P. Reeves et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Innate Immunity in the Respiratory Epithelium
- (2011) Dane Parker et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Innate Inflammatory Responses of Pediatric Cystic Fibrosis Airway Epithelial Cells
- (2011) Erika N. Sutanto et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Induction of Type I Interferon Signaling byPseudomonas aeruginosaIs Diminished in Cystic Fibrosis Epithelial Cells
- (2011) Dane Parker et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Staphylococcus aureus Biofilm and Planktonic cultures differentially impact gene expression, mapk phosphorylation, and cytokine production in human keratinocytes
- (2011) Patrick R Secor et al. BMC MICROBIOLOGY
- Inflammatory Responses to Individual Microorganisms in the Lungs of Children With Cystic Fibrosis
- (2011) C. Gangell et al. CLINICAL INFECTIOUS DISEASES
- Lack of an exaggerated inflammatory response on virus infection in cystic fibrosis
- (2011) E. Kieninger et al. EUROPEAN RESPIRATORY JOURNAL
- Infection of Polarized Airway Epithelial Cells by Normal and Small-Colony Variant Strains of Staphylococcus aureus Is Increased in Cells with Abnormal Cystic Fibrosis Transmembrane Conductance Regulator Function and Is Influenced by NF-κB
- (2011) Gabriel Mitchell et al. INFECTION AND IMMUNITY
- Anaerobiosis-Induced Loss of Cytotoxicity Is Due to Inactivation of Quorum Sensing in Pseudomonas aeruginosa
- (2011) Kang-Mu Lee et al. INFECTION AND IMMUNITY
- Strategies of professional phagocytes in vivo: unlike macrophages, neutrophils engulf only surface-associated microbes
- (2011) E. Colucci-Guyon et al. JOURNAL OF CELL SCIENCE
- Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection
- (2011) Maurizio Renna et al. JOURNAL OF CLINICAL INVESTIGATION
- Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis
- (2011) E. M. Bruscia et al. JOURNAL OF IMMUNOLOGY
- IL-17A and TNF- Exert Synergistic Effects on Expression of CXCL5 by Alveolar Type II Cells In Vivo and In Vitro
- (2011) Y. Liu et al. JOURNAL OF IMMUNOLOGY
- Mechanisms controlling Th17 cytokine expression and host defense
- (2011) Jeremy P. McAleer et al. JOURNAL OF LEUKOCYTE BIOLOGY
- A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation
- (2011) Bonnie W. Ramsey et al. NEW ENGLAND JOURNAL OF MEDICINE
- The F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs
- (2011) L. S. Ostedgaard et al. Science Translational Medicine
- Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation
- (2011) J P Clancy et al. THORAX
- Streptococcus pneumoniae DNA Initiates Type I Interferon Signaling in the Respiratory Tract
- (2011) D. Parker et al. mBio
- Acute inhalation of hypertonic saline does not improve mucociliary clearance in all children with cystic fibrosis
- (2011) Beth L Laube et al. BMC Pulmonary Medicine
- A Pseudomonas aeruginosa Toxin that Hijacks the Host Ubiquitin Proteolytic System
- (2011) Jennifer M. Bomberger et al. PLoS Pathogens
- AAV Exploits Subcellular Stress Associated with Inflammation, Endoplasmic Reticulum Expansion, and Misfolded Proteins in Models of Cystic Fibrosis
- (2011) Jarrod S. Johnson et al. PLoS Pathogens
- MAPK signaling pathways regulate IL-8 mRNA stability and IL-8 protein expression in cystic fibrosis lung epithelial cell lines
- (2010) Sharmistha Bhattacharyya et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Denufosol Tetrasodium in Patients with Cystic Fibrosis and Normal to Mildly Impaired Lung Function
- (2010) Frank J. Accurso et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Lack of Cystic Fibrosis Transmembrane Conductance Regulator in CD3+Lymphocytes Leads to Aberrant Cytokine Secretion and Hyperinflammatory Adaptive Immune Responses
- (2010) Christian Mueller et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia
- (2010) Jeng-Haur Chen et al. CELL
- The Changing Microbial Epidemiology in Cystic Fibrosis
- (2010) J. J. LiPuma CLINICAL MICROBIOLOGY REVIEWS
- Early production of IL-17 protects against acute pulmonaryPseudomonas aeruginosainfection in mice
- (2010) Jialin Liu et al. FEMS IMMUNOLOGY AND MEDICAL MICROBIOLOGY
- Effect of Azithromycin on Pulmonary Function in Patients With Cystic Fibrosis Uninfected With Pseudomonas aeruginosaA Randomized Controlled Trial
- (2010) Lisa Saiman JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
- Association Between Respiratory Tract Methicillin-Resistant Staphylococcus aureus and Survival in Cystic Fibrosis
- (2010) Elliott C. Dasenbrook JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
- Airway Surface Liquid Volume Regulation Determines Different Airway Phenotypes in Liddle Compared with βENaC-overexpressing Mice
- (2010) Marcus A. Mall et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Enhances Activation of p38 and ERK MAPKs, Increasing Interleukin-6 Synthesis in Airway Epithelial Cells Exposed toPseudomonas aeruginosa
- (2010) Julie Bérubé et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Pulmonary Proteases in the Cystic Fibrosis Lung Induce Interleukin 8 Expression from Bronchial Epithelial Cells via a Heme/Meprin/Epidermal Growth Factor Receptor/Toll-like Receptor Pathway
- (2010) Sonya Cosgrove et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis
- (2010) Xingshen Sun et al. JOURNAL OF CLINICAL INVESTIGATION
- Vitamin D3 attenuates Th2 responses to Aspergillus fumigatus mounted by CD4+ T cells from cystic fibrosis patients with allergic bronchopulmonary aspergillosis
- (2010) James L. Kreindler et al. JOURNAL OF CLINICAL INVESTIGATION
- The Streptococcus milleri Population of a Cystic Fibrosis Clinic Reveals Patient Specificity and Intraspecies Diversity
- (2010) C. D. Sibley et al. JOURNAL OF CLINICAL MICROBIOLOGY
- Inhaled mannitol in patients with cystic fibrosis: A randomised open-label dose response trial
- (2010) Alejandro Teper et al. Journal of Cystic Fibrosis
- IFN Regulatory Factor 3 Contributes to the Host Response during Pseudomonas aeruginosa Lung Infection in Mice
- (2010) S. O. Carrigan et al. JOURNAL OF IMMUNOLOGY
- Extracellular DNA: A Major Proinflammatory Component of Pseudomonas aeruginosa Biofilms
- (2010) J. I. Fuxman Bass et al. JOURNAL OF IMMUNOLOGY
- In Cystic Fibrosis Homozygotes and Heterozygotes, Neutrophil Apoptosis Is Delayed and Modulated by Diamide or Roscovitine: Evidence for an Innate Neutrophil Disturbance
- (2010) Sandra Moriceau et al. Journal of Innate Immunity
- Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition
- (2010) Alessandro Luciani et al. NATURE CELL BIOLOGY
- Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation
- (2010) Frank J. Accurso et al. NEW ENGLAND JOURNAL OF MEDICINE
- Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth
- (2010) D. A. Stoltz et al. Science Translational Medicine
- Parallel Evolution in Pseudomonas aeruginosa over 39,000 Generations In Vivo
- (2010) H. K. Huse et al. mBio
- Airway surface liquid depth measured in ex vivo fragments of pig and human trachea: dependence on Na+ and Cl− channel function
- (2009) Yuanlin Song et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Pseudomonas aeruginosaMicroevolution during Cystic Fibrosis Lung Infection Establishes Clones with Adapted Virulence
- (2009) Alessandra Bragonzi et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- TLR-4–Mediated Innate Immunity Is Reduced in Cystic Fibrosis Airway Cells
- (2009) Gerrit John et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Impaired Interleukin-8 Chemokine Secretion byStaphylococcus aureus–Activated Epithelium and T-Cell Chemotaxis in Cystic Fibrosis
- (2009) Denise Al Alam et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Biofilm-forming Pseudomonas aeruginosa bacteria undergo lipopolysaccharide structural modifications and induce enhanced inflammatory cytokine response in human monocytes
- (2009) Cristina D. Ciornei et al. Innate Immunity
- Coronin-1 Is Associated with Neutrophil Survival and Is Cleaved during Apoptosis: Potential Implication in Neutrophils from Cystic Fibrosis Patients
- (2009) S. Moriceau et al. JOURNAL OF IMMUNOLOGY
- Decreased Levels of Secretory Leucoprotease Inhibitor in the Pseudomonas-Infected Cystic Fibrosis Lung Are Due to Neutrophil Elastase Degradation
- (2009) S. Weldon et al. JOURNAL OF IMMUNOLOGY
- Dynamics of Adaptive Microevolution of HypermutablePseudomonas aeruginosaduring Chronic Pulmonary Infection in Patients with Cystic Fibrosis
- (2009) Christina Hoboth et al. JOURNAL OF INFECTIOUS DISEASES
- Respiratory syncytial virus infection facilitates acute colonization ofPseudomonas aeruginosain mice
- (2009) Angélica M.M. de Vrankrijker et al. JOURNAL OF MEDICAL VIROLOGY
- Pseudomonas aeruginosabiofilms in the respiratory tract of cystic fibrosis patients
- (2009) Thomas Bjarnsholt et al. PEDIATRIC PULMONOLOGY
- CFTR Is a Negative Regulator of NFκB Mediated Innate Immune Response
- (2009) Neeraj Vij 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
- Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
- (2009) F. Van Goor et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Cystic Fibrosis Transmembrane Conductance Regulator Controls Lung Proteasomal Degradation and Nuclear Factor-κB Activity in Conditions of Oxidative Stress
- (2008) Emilie Boncoeur et al. AMERICAN JOURNAL OF PATHOLOGY
- Novel human bronchial epithelial cell lines for cystic fibrosis research
- (2008) M. L. Fulcher et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/−Mice
- (2008) Emanuela M. Bruscia et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Oxidative Stress CausesIL8Promoter Hyperacetylation in Cystic Fibrosis Airway Cell Models
- (2008) Toni R. Bartling et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Oxidative stress caused by pyocyanin impairs CFTR Cl− transport in human bronchial epithelial cells
- (2008) Christian Schwarzer et al. FREE RADICAL BIOLOGY AND MEDICINE
- Production of CFTR-null and CFTR-ΔF508 heterozygous pigs by adeno-associated virus–mediated gene targeting and somatic cell nuclear transfer
- (2008) Christopher S. Rogers et al. JOURNAL OF CLINICAL INVESTIGATION
- Anti-inflammatory effect of miglustat in bronchial epithelial cells
- (2008) Maria Cristina Dechecchi et al. Journal of Cystic Fibrosis
- The role of chloride anion and CFTR in killing of Pseudomonas aeruginosa by normal and CF neutrophils
- (2008) R. G. Painter et al. JOURNAL OF LEUKOCYTE BIOLOGY
- Relationship between Inhaled Corticosteroid Therapy and Rate of Lung Function Decline in Children with Cystic Fibrosis
- (2008) Clement L. Ren et al. JOURNAL OF PEDIATRICS
- TRAM couples endocytosis of Toll-like receptor 4 to the induction of interferon-β
- (2008) Jonathan C Kagan et al. NATURE IMMUNOLOGY
- Bronchial provocation testing with dry powder mannitol in children with cystic fibrosis
- (2008) Caro Minasian et al. PEDIATRIC PULMONOLOGY
- Prevalence and Impact of Respiratory Viral Infections in Young Children With Cystic Fibrosis: Prospective Cohort Study
- (2008) B. E. van Ewijk et al. PEDIATRICS
- Prolonged survival of neutrophils from patients with F508 CFTR mutations
- (2008) D J McKeon et al. THORAX
- Pharmacoscintigraphic and pharmacokinetic evaluation of tobramycin DPI formulations in cystic fibrosis patients
- (2007) Gabrielle Pilcer et al. EUROPEAN JOURNAL OF PHARMACEUTICS AND BIOPHARMACEUTICS
Publish scientific posters with Peeref
Peeref publishes scientific posters from all research disciplines. Our Diamond Open Access policy means free access to content and no publication fees for authors.
Learn MoreAsk a Question. Answer a Question.
Quickly pose questions to the entire community. Debate answers and get clarity on the most important issues facing researchers.
Get Started