Published in J Cyst Fibros on August 01, 2004
Emergent properties of proteostasis in managing cystic fibrosis. Cold Spring Harb Perspect Biol (2011) 1.24
A morphometric study of mucins and small airway plugging in cystic fibrosis. Thorax (2006) 1.16
Suppression of premature termination codons as a therapeutic approach. Crit Rev Biochem Mol Biol (2012) 1.13
Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice. Respir Res (2006) 1.12
Species-specific differences in mouse and human airway epithelial biology of recombinant adeno-associated virus transduction. Am J Respir Cell Mol Biol (2005) 1.12
Loss of CFTR affects biliary epithelium innate immunity and causes TLR4-NF-κB-mediated inflammatory response in mice. Gastroenterology (2011) 1.07
Comparative biology of rAAV transduction in ferret, pig and human airway epithelia. Gene Ther (2007) 0.96
Tissue and cellular expression patterns of porcine CFTR: similarities to and differences from human CFTR. J Histochem Cytochem (2010) 0.91
Genomic, tissue expression, and protein characterization of pCLCA1, a putative modulator of cystic fibrosis in the pig. J Histochem Cytochem (2009) 0.91
Attenuation of host defense function of lung phagocytes in young cystic fibrosis patients. J Cyst Fibros (2005) 0.88
Efficient gene transfer into the mouse lung by fetal intratracheal injection of rAAV2/6.2. Mol Ther (2010) 0.86
Transcription-dependent spatial arrangements of CFTR and conserved adjacent loci are not conserved in human and murine nuclei. Chromosoma (2008) 0.80
Pancreatic pathophysiology in cystic fibrosis. J Pathol (2015) 0.79
The fetal respiratory system as target for antenatal therapy. Facts Views Vis Obgyn (2011) 0.79
Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function. PLoS One (2013) 0.79
Regulation of hepatic sulfotransferase (SULT) 1E1 expression and effects on estrogenic activity in cystic fibrosis (CF). J Steroid Biochem Mol Biol (2009) 0.79
The porcine chloride channel calcium-activated family member pCLCA4a mirrors lung expression of the human hCLCA4. J Histochem Cytochem (2012) 0.78
Influence of neutrophil defects on Burkholderia cepacia complex pathogenesis. Front Cell Infect Microbiol (2011) 0.78
Potential of anti-inflammatory treatment for cystic fibrosis lung disease. J Inflamm Res (2010) 0.78
Barriers to inhaled gene therapy of obstructive lung diseases: A review. J Control Release (2016) 0.77
Expression of S100A8 correlates with inflammatory lung disease in congenic mice deficient of the cystic fibrosis transmembrane conductance regulator. Respir Res (2006) 0.76
Naturally Occurring Deletion Mutants of the Pig-Specific, Intestinal Crypt Epithelial Cell Protein CLCA4b without Apparent Phenotype. PLoS One (2015) 0.75
Cholic acid induces a Cftr dependent biliary secretion and liver growth response in mice. PLoS One (2015) 0.75
Characterisation of electrogenic nutrient absorption in the Cftr TgH(neoim)Hgu mouse model. J Comp Physiol B (2008) 0.75
A randomized controlled trial of peripheral blood mononuclear cell depletion in experimental human lung inflammation. Am J Respir Crit Care Med (2013) 2.71
The human antimicrobial peptide LL-37 is a multifunctional modulator of innate immune responses. J Immunol (2002) 2.57
Impact of LL-37 on anti-infective immunity. J Leukoc Biol (2004) 2.01
Diagnostic importance of pulmonary interleukin-1beta and interleukin-8 in ventilator-associated pneumonia. Thorax (2009) 1.87
Monocytes control second-phase neutrophil emigration in established lipopolysaccharide-induced murine lung injury. Am J Respir Crit Care Med (2012) 1.82
A re-evaluation of the role of host defence peptides in mammalian immunity. Curr Protein Pept Sci (2005) 1.78
Immunomodulatory activities of small host defense peptides. Antimicrob Agents Chemother (2005) 1.67
Activation of intestinal Cl- secretion by lubiprostone requires the cystic fibrosis transmembrane conductance regulator. Gastroenterology (2009) 1.59
The role of the purinergic P2X7 receptor in inflammation. J Inflamm (Lond) (2007) 1.50
Electrophysiological studies of malaria parasite-infected erythrocytes: current status. Int J Parasitol (2007) 1.47
The human cationic peptide LL-37 induces activation of the extracellular signal-regulated kinase and p38 kinase pathways in primary human monocytes. J Immunol (2004) 1.41
Antiviral activity and increased host defense against influenza infection elicited by the human cathelicidin LL-37. PLoS One (2011) 1.39
Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine. Mol Pharmacol (2010) 1.36
Thrombospondin-1 mimetic peptide inhibitors of angiogenesis and tumor growth: design, synthesis, and optimization of pharmacokinetics and biological activities. J Med Chem (2005) 1.31
Human beta-defensin 3 has immunosuppressive activity in vitro and in vivo. Eur J Immunol (2010) 1.26
The human cationic host defense peptide LL-37 mediates contrasting effects on apoptotic pathways in different primary cells of the innate immune system. J Leukoc Biol (2006) 1.25
Interaction and cellular localization of the human host defense peptide LL-37 with lung epithelial cells. Infect Immun (2005) 1.24
Dying and necrotic neutrophils are anti-inflammatory secondary to the release of alpha-defensins. J Immunol (2009) 1.21
Plasmodium falciparum-activated chloride channels are defective in erythrocytes from cystic fibrosis patients. J Biol Chem (2003) 1.20
RNA:DNA hybrids are a novel molecular pattern sensed by TLR9. EMBO J (2014) 1.19
The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells. Bone (2009) 1.18
Primary immunodeficiency to pneumococcal infection due to a defect in Toll-like receptor signaling. J Pediatr (2004) 1.12
Differential persistence among genomovars of the Burkholderia cepacia complex in a murine model of pulmonary infection. Infect Immun (2002) 1.07
Plasminogen kringle 5 induces apoptosis of brain microvessel endothelial cells: sensitization by radiation and requirement for GRP78 and LRP1. Cancer Res (2009) 1.05
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat. FEBS Lett (2006) 1.04
Prevalence of Toll-like receptor signalling defects in apparently healthy children who developed invasive pneumococcal infection. Clin Immunol (2006) 1.03
Apoptosis of airway epithelial cells: human serum sensitive induction by the cathelicidin LL-37. Am J Respir Cell Mol Biol (2005) 1.02
C5a mediates peripheral blood neutrophil dysfunction in critically ill patients. Am J Respir Crit Care Med (2009) 1.01
C5a-mediated neutrophil dysfunction is RhoA-dependent and predicts infection in critically ill patients. Blood (2011) 0.98
The hematopoietic system-specific minor histocompatibility antigen HA-1 shows aberrant expression in epithelial cancer cells. J Exp Med (2002) 0.96
Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells. J Pharmacol Exp Ther (2008) 0.96
The human cathelicidin LL-37 preferentially promotes apoptosis of infected airway epithelium. Am J Respir Cell Mol Biol (2010) 0.93
Secondary necrosis of apoptotic neutrophils induced by the human cathelicidin LL-37 is not proinflammatory to phagocytosing macrophages. J Leukoc Biol (2009) 0.93
Trappin-2 promotes early clearance of Pseudomonas aeruginosa through CD14-dependent macrophage activation and neutrophil recruitment. Am J Pathol (2009) 0.92
Cationic host defence peptides: potential as antiviral therapeutics. BioDrugs (2013) 0.92
The human cathelicidin LL-37 has antiviral activity against respiratory syncytial virus. PLoS One (2013) 0.88
Rescue of epithelial HCO3- secretion in murine intestine by apical membrane expression of the cystic fibrosis transmembrane conductance regulator mutant F508del. J Physiol (2012) 0.88
Spontaneous rescue from cystic fibrosis in a mouse model. BMC Genet (2006) 0.87
Antiviral potential of cathelicidins. Future Microbiol (2014) 0.85
The host defense peptide LL-37 selectively permeabilizes apoptotic leukocytes. Antimicrob Agents Chemother (2008) 0.85
Immunohistochemistry of CFTR in native tissues and primary epithelial cell cultures. J Cyst Fibros (2004) 0.84
An integrated model for developing research skills in an undergraduate medical curriculum: appraisal of an approach using student selected components. Perspect Med Educ (2013) 0.84
Proteomic analysis of naphthalene-induced airway epithelial injury and repair in a cystic fibrosis mouse model. J Proteome Res (2009) 0.84
Stimulation of salivary secretion in vivo by CFTR potentiators in Cftr+/+ and Cftr-/- mice. J Cyst Fibros (2007) 0.83
Pseudomonas aeruginosa: role in the pathogenesis of the CF lung lesion. Semin Respir Crit Care Med (2003) 0.83
Efficient induction of minor histocompatibility antigen HA-1-specific cytotoxic T-cells using dendritic cells retrovirally transduced with HA-1-coding cDNA. Biol Blood Marrow Transplant (2002) 0.83
Exclusive TCRVbeta chain usage of ex vivo generated minor Histocompatibility antigen HA-1 specific cytotoxic T cells: implications for monitoring of immunotherapy of leukemia by TCRBV spectratyping. Hematol J (2002) 0.83
Urine electrolyte, mineral, and protein excretion in NHERF-2 and NHERF-1 null mice. Am J Physiol Renal Physiol (2008) 0.82
Persistence of Burkholderia multivorans within the pulmonary macrophage in the murine lung. Infect Immun (2004) 0.82
Recipient cytokine genotypes for TNF-alpha and IL-10 and the minor histocompatibility antigens HY and CD31 codon 125 are not associated with occurrence or severity of acute GVHD in unrelated cord blood transplantation: a retrospective analysis. Transplantation (2002) 0.79
Very mild disease phenotype of congenic CftrTgH(neoim)Hgu cystic fibrosis mice. BMC Genet (2008) 0.78
Osteopenia in Cftr-deltaF508 mice. J Cyst Fibros (2010) 0.78
Expression of intestinal and lung alkaline sphingomyelinase and neutral ceramidase in cystic fibrosis f508del transgenic mice. J Pediatr Gastroenterol Nutr (2008) 0.77
Lysyl 4-aminobenzoic acid derivatives as potent small molecule mimetics of plasminogen kringle 5. Bioorg Med Chem Lett (2004) 0.77
Robust TLR4-induced gene expression patterns are not an accurate indicator of human immunity. J Transl Med (2010) 0.77
Letting the CAThelicidin out of the bag, as a therapeutic modulator of the adaptive immune system. Leuk Res (2005) 0.75
Correction: Human β-D-3 Exacerbates MDA5 but Suppresses TLR3 Responses to the Viral Molecular Pattern Mimic Polyinosinic:Polycytidylic Acid. PLoS Genet (2016) 0.75
Reply: The alveolar macrophage and acute respiratory distress syndrome: a silent actor? Am J Respir Crit Care Med (2014) 0.75
Knee Arthroscopy Simulation: A Randomized Controlled Trial Evaluating the Effectiveness of the Imperial Knee Arthroscopy Cognitive Task Analysis (IKACTA) Tool. J Bone Joint Surg Am (2017) 0.75