Lessons learned from the cystic fibrosis pig.
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[1] D. Meyerholz,et al. Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans , 2016, Toxicologic pathology.
[2] D. Meyerholz,et al. Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility. , 2016, American journal of respiratory and critical care medicine.
[3] D. Meyerholz,et al. Pancreatic pathophysiology in cystic fibrosis , 2016, The Journal of pathology.
[4] J. Wine,et al. Airway Gland Structure and Function. , 2015, Physiological reviews.
[5] Xiaohong Huang,et al. Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. , 2015, The New England journal of medicine.
[6] I. Callebaut,et al. Impact of the F508del mutation on ovine CFTR, a Cl− channel with enhanced conductance and ATP‐dependent gating , 2015, The Journal of physiology.
[7] D. Meyerholz,et al. Sonographic evidence of abnormal tracheal cartilage ring structure in cystic fibrosis , 2015, The Laryngoscope.
[8] D. Meyerholz,et al. Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. , 2015, American journal of physiology. Gastrointestinal and liver physiology.
[9] D. Meyerholz,et al. Origins of cystic fibrosis lung disease. , 2015, The New England journal of medicine.
[10] M. Welsh,et al. pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37 , 2014, Proceedings of the National Academy of Sciences.
[11] Garry R. Cutting,et al. Cystic fibrosis genetics: from molecular understanding to clinical application , 2014, Nature Reviews Genetics.
[12] D. Stoltz,et al. Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF , 2014, Pediatric pulmonology.
[13] D. Meyerholz,et al. Development and translational imaging of a TP53 porcine tumorigenesis model. , 2014, The Journal of clinical investigation.
[14] M. Welsh,et al. A genomic signature approach to rescue ΔF508-cystic fibrosis transmembrane conductance regulator biosynthesis and function. , 2014, American journal of respiratory cell and molecular biology.
[15] E. Hoffman,et al. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis , 2014, Science.
[16] G. Lukács,et al. Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression , 2014, Science Translational Medicine.
[17] D. Stoltz,et al. Genotype-specific alterations in vascular smooth muscle cell function in cystic fibrosis piglets. , 2014, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
[18] K. Chu,et al. Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats , 2014, PloS one.
[19] E. Hoffman,et al. Air trapping and airflow obstruction in newborn cystic fibrosis piglets. , 2013, American journal of respiratory and critical care medicine.
[20] E. Hoffman,et al. Early Airway Structural Changes in Cystic Fibrosis Pigs as a Determinant of Particle Distribution and Deposition , 2013, Annals of Biomedical Engineering.
[21] E. Hoffman,et al. Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs. , 2013, The Journal of clinical investigation.
[22] T. Moninger,et al. Adenoviral gene transfer corrects the ion transport defect in the sinus epithelia of a porcine CF model. , 2013, Molecular therapy : the journal of the American Society of Gene Therapy.
[23] M. Bagnat,et al. Cftr controls lumen expansion and function of Kupffer’s vesicle in zebrafish , 2013, Development.
[24] M. Welsh,et al. CFTR-deficient pigs display peripheral nervous system defects at birth , 2013, Proceedings of the National Academy of Sciences.
[25] D. Meyerholz,et al. Sinus Hypoplasia Precedes Sinus Infection in a Porcine Model of Cystic Fibrosis , 2012, The Laryngoscope.
[26] E. Wolf,et al. Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis , 2012, Journal of Molecular Medicine.
[27] M. Welsh,et al. Reduced Airway Surface pH Impairs Bacterial Killing in the Porcine Cystic Fibrosis Lung , 2012, Nature.
[28] F. Clubb,et al. Swine as Models in Biomedical Research and Toxicology Testing , 2012, Veterinary pathology.
[29] B. Schultz,et al. Swine Models of Cystic Fibrosis Reveal Male Reproductive Tract Phenotype at Birth1 , 2011, Biology of reproduction.
[30] M. Welsh,et al. Human cystic fibrosis airway epithelia have reduced Cl− conductance but not increased Na+ conductance , 2011, Proceedings of the National Academy of Sciences.
[31] S. Richter,et al. The ΔF508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis–Like Disease in Pigs , 2011, Science Translational Medicine.
[32] M. Welsh,et al. Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia , 2010, Cell.
[33] G. McLennan,et al. Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. , 2010, American journal of respiratory and critical care medicine.
[34] M. Welsh,et al. Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth , 2010, Proceedings of the National Academy of Sciences.
[35] N. Joo,et al. Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs. , 2010, The Journal of clinical investigation.
[36] N. Joo,et al. Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. , 2010, The Journal of clinical investigation.
[37] J. Wine. The Development of Lung Disease in Cystic Fibrosis Pigs , 2010, Science Translational Medicine.
[38] S. Richter,et al. Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth , 2010, Science Translational Medicine.
[39] E. Wolf,et al. Transgenic pigs as models for translational biomedical research , 2010, Journal of Molecular Medicine.
[40] D. Meyerholz,et al. Pathology of gastrointestinal organs in a porcine model of cystic fibrosis. , 2010, The American journal of pathology.
[41] D. Meyerholz,et al. Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs , 2008, Science.
[42] J. Bernaudin,et al. Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator‐deficient mice , 2008, The Journal of physiology.
[43] J. T. Fisher,et al. The porcine lung as a potential model for cystic fibrosis. , 2008, American journal of physiology. Lung cellular and molecular physiology.
[44] D. Meyerholz,et al. Collectins and Cationic Antimicrobial Peptides of the Respiratory Epithelia , 2006, Veterinary pathology.
[45] J. Grigg,et al. New insights into pulmonary inflammation in cystic fibrosis , 2006, Archives of Disease in Childhood.
[46] S. Ballard,et al. Liquid secretion properties of airway submucosal glands , 2004, The Journal of physiology.
[47] R. Rowntree,et al. The Phenotypic Consequences of CFTR Mutations , 2003, Annals of human genetics.
[48] F. Ratjen,et al. Cystic fibrosis , 2003, The Lancet.
[49] A. Webster,et al. Effects of Ammonia Inhalation and Acetic Acid Pretreatment on Colonization Kinetics of Toxigenic Pasteurella multocida within Upper Respiratory Tracts of Swine , 1998, Journal of Clinical Microbiology.
[50] L. Tsui,et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. , 1989, Science.
[51] J. Sturgess,et al. Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants. , 1982, The American journal of pathology.
[52] J. A. Fraser Roberts,et al. Fibrocystic Disease of the Pancreas: A Congenital Disorder of Mucus Production—Mucosis , 1953 .
[53] D. Andersen. CYSTIC FIBROSIS OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE: A CLINICAL AND PATHOLOGIC STUDY , 1938 .
[54] D. Meyerholz,et al. Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass. , 2015, Clinical science.
[55] D. Meyerholz,et al. CFTR in Sarcoplasmic Reticulum of Airway Smooth Muscle: Implications for Airway Contractility , 2015 .
[56] P. Halbur,et al. Exogenous porcine viruses. , 2003, Current topics in microbiology and immunology.
[57] R. Boucher,et al. Pathophysiology of gene-targeted mouse models for cystic fibrosis. , 1999, Physiological reviews.
[58] A. Baskerville. Histological and ultrastructural observations on the development of the lung of the fetal pig. , 1976, Acta anatomica.
[59] Esterly,et al. Pathology of cystic fibrosis review of the literature and comparison with 146 autopsied cases. , 1975, Perspectives in pediatric pathology.