Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation

Infection Is Not Required for Mucoinflammatory Lung Disease in CFTR-Knockout Ferrets

(2018) Bradley H. Rosen et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Platelet Depletion Impairs Host Defense to Pulmonary Infection with Pseudomonas aeruginosa in Mice

(2018) Richard T. Amison et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Start a fire, kill the bug: The role of platelets in inflammation and infection

(2018) Carsten Deppermann et al.   Innate Immunity

The lung is a site of platelet biogenesis and a reservoir for haematopoietic progenitors

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Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

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Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

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Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis

(2017) Robert D Gray et al.   THORAX

Cystic fibrosis

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Directed transport of neutrophil-derived extracellular vesicles enables platelet-mediated innate immune response

(2016) Jan Rossaint et al.   Nature Communications

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

Ion Channels in Innate and Adaptive Immunity

(2015) Stefan Feske et al.   Annual Review of Immunology

Essential role of class II PI3K-C2  in platelet membrane morphology

(2015) C. Valet et al.   BLOOD

TRPC6 is the endothelial calcium channel that regulates leukocyte transendothelial migration during the inflammatory response

(2015) Evan W. Weber et al.   JOURNAL OF EXPERIMENTAL MEDICINE

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

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

A Critical Role for the Transient Receptor Potential Channel Type 6 in Human Platelet Activation

(2015) Hari Priya Vemana et al.   PLoS One

TRPC6 participates in the regulation of cytosolic basal calcium concentration in murine resting platelets

(2014) Letizia Albarran et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Aspirin-triggered 15-epi-lipoxin A4 regulates neutrophil-platelet aggregation and attenuates acute lung injury in mice

(2014) G. Ortiz-Munoz et al.   BLOOD

Rate of improvement of CF life expectancy exceeds that of general population—Observational death registration study

(2014) Matthew N. Hurley et al.   Journal of Cystic Fibrosis

The Pseudomonas aeruginosa Type III Translocon Is Required for Biofilm Formation at the Epithelial Barrier

(2014) Cindy S. Tran et al.   PLoS Pathogens

Human inflammatory and resolving lipid mediator responses to resistance exercise and ibuprofen treatment

(2013) James F. Markworth et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

Effect of VX-770 (Ivacaftor) and OAG on Ca2+ influx and CFTR activity in G551D and F508del-CFTR expressing cells

(2013) Laura Vachel et al.   Journal of Cystic Fibrosis

Risk Factors for Bronchiectasis in Children with Cystic Fibrosis

(2013) Peter D. Sly et al.   NEW ENGLAND JOURNAL OF MEDICINE

Important Role of Platelets in Modulating Endotoxin-Induced Lung Inflammation in CFTR-Deficient Mice

(2013) Caiqi Zhao et al.   PLoS One

Platelets induce neutrophil extracellular traps in transfusion-related acute lung injury

(2012) Axelle Caudrillier et al.   JOURNAL OF CLINICAL INVESTIGATION

TLR4 activation of TRPC6-dependent calcium signaling mediates endotoxin-induced lung vascular permeability and inflammation

(2012) Mohammad Tauseef et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Mouse transient receptor potential channel 6: Role in hemostasis and thrombogenesis

(2011) Enma V. Paez Espinosa et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

(2011) Xiao Su et al.   INFLAMMATION RESEARCH

Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis

(2011) E. M. Bruscia et al.   JOURNAL OF IMMUNOLOGY

Potent, Metabolically Stable Benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR Inhibitors for Polycystic Kidney Disease

(2011) David S. Snyder et al.   JOURNAL OF MEDICINAL CHEMISTRY

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

Novel Anti-bacterial Activities of β-defensin 1 in Human Platelets: Suppression of Pathogen Growth and Signaling of Neutrophil Extracellular Trap Formation

(2011) Bjoern F. Kraemer et al.   PLoS Pathogens

Transient Receptor Potential Canonical Channel 6 Links Ca2+Mishandling to Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Cystic Fibrosis

(2010) Fabrice Antigny et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

The transient receptor potential channel antagonist SKF96365 is a potent blocker of low-voltage-activated T-type calcium channels

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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

CFTR-mediated halide transport in phagosomes of human neutrophils

(2010) Richard G. Painter et al.   JOURNAL OF LEUKOCYTE BIOLOGY

Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth

(2010) D. A. Stoltz et al.   Science Translational Medicine

Neutrophil-specific deletion of Syk kinase results in reduced host defense to bacterial infection

(2009) J. A. Van Ziffle et al.   BLOOD

Platelet depletion and aspirin treatment protect mice in a two-event model of transfusion-related acute lung injury

(2009) Mark R. Looney et al.   JOURNAL OF CLINICAL INVESTIGATION

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

Generation of a conditional null allele forCftrin mice

(2008) Craig A. Hodges et al.   GENESIS

Store-operated Ca2+ entry in platelets occurs independently of transient receptor potential (TRP) C1

(2008) David Varga-Szabo et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs

(2008) C. S. Rogers et al.   SCIENCE