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

Effects of Liver X Receptor Agonist Treatment on Pulmonary Inflammation and Host Defense

(2014) K. Smoak et al.   JOURNAL OF IMMUNOLOGY

Bioelectric Characterization of Epithelia from NeonatalCFTRKnockout Ferrets

(2013) John T. Fisher et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Optimal Complement-Mediated Phagocytosis ofPseudomonas aeruginosaby Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator–Dependent

(2013) Pauline B. Van de Weert–van Leeuwen et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Specific cleavage of the lung surfactant protein A by human cathepsin S may impair its antibacterial properties

(2013) Fabien Lecaille et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Low Levels of Insulin-like Growth Factor-1 Contribute to Alveolar Macrophage Dysfunction in Cystic Fibrosis

(2013) J. L. Bessich et al.   JOURNAL OF IMMUNOLOGY

Method for Quantitative Study of Airway Functional Microanatomy Using Micro-Optical Coherence Tomography

(2013) Linbo Liu et al.   PLoS One

Immune Responses in Cystic Fibrosis

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

Airway Defense Mechanisms

(2012) Grant W. Waterer   CLINICS IN CHEST MEDICINE

Eukaryotic Initiation Factor 2 (eIF2) Signaling Regulates Proinflammatory Cytokine Expression and Bacterial Invasion

(2012) Niraj Shrestha et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Innate immunity in cystic fibrosis lung disease

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

Inhibition of Mammalian Target of Rapamycin Augments Lipopolysaccharide-Induced Lung Injury and Apoptosis

(2012) J. A. Fielhaber et al.   JOURNAL OF IMMUNOLOGY

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

(2012) Alejandro A. Pezzulo et al.   NATURE

Cystic fibrosis: a mucosal immunodeficiency syndrome

(2012) Taylor Sitarik Cohen et al.   NATURE MEDICINE

Innate Immunity in the Respiratory Epithelium

(2011) Dane Parker et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Innate immune responses to Pseudomonas aeruginosa infection

(2011) Elise G. Lavoie et al.   MICROBES AND INFECTION

Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis

(2011) S. C. Ranganathan et al.   THORAX

Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia

(2010) Jeng-Haur Chen et al.   CELL

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

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

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

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

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

Mapping the Lung Proteome in Cystic Fibrosis

(2009) Sina A. Gharib et al.   JOURNAL OF PROTEOME RESEARCH

Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis

(2009) Stacey L. Peterson-Carmichael et al.   PEDIATRIC PULMONOLOGY

Adeno-associated virus–targeted disruption of the CFTR gene in cloned ferrets

(2008) Xingshen Sun et al.   JOURNAL OF CLINICAL INVESTIGATION

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

Alterations in immune response and PPAR/LXR regulation in cystic fibrosis macrophages

(2007) Charlotte Andersson et al.   Journal of Cystic Fibrosis