Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology

Origins of Cystic Fibrosis Lung Disease

(2015) David A. Stoltz et al.   NEW ENGLAND JOURNAL OF MEDICINE

Gastrointestinal Pathology in Juvenile and Adult CFTR-Knockout Ferrets

(2014) Xingshen Sun et al.   AMERICAN JOURNAL OF PATHOLOGY

Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes

(2014) L. Leeuwen et al.   ARCHIVES OF DISEASE IN CHILDHOOD

Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: A double blind prospective study

(2014) Rosa del Campo et al.   Journal of Cystic Fibrosis

Models: stretching the skills of cell lines and mice

(2014) Vivien Marx   NATURE METHODS

Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats

(2014) Katherine L. Tuggle et al.   PLoS One

pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37

(2014) Mahmoud H. Abou Alaiwa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Organogenesis in a dish: Modeling development and disease using organoid technologies

(2014) M. A. Lancaster et al.   SCIENCE

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

(2014) M. J. Hoegger et al.   SCIENCE

Effect of esomeprazole versus placebo on pulmonary exacerbations in cystic fibrosis

(2014) Emily DiMango et al.   BMC Pulmonary Medicine

Bioelectric Characterization of Epithelia from NeonatalCFTRKnockout Ferrets

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

Lung Phenotype of Juvenile and Adult Cystic Fibrosis Transmembrane Conductance Regulator–Knockout Ferrets

(2013) Xingshen Sun et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Distal intestinal obstruction syndrome in cystic fibrosis: presentation, outcome and management in a tertiary hospital (2007-2012)

(2013) Rami Subhi et al.   ANZ JOURNAL OF SURGERY

Children with Cystic Fibrosis Have Prolonged Chemical Clearance of Acid Reflux Compared to Symptomatic Children Without Cystic Fibrosis

(2013) Frederick W. Woodley et al.   DIGESTIVE DISEASES AND SCIENCES

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs

(2013) David A. Stoltz et al.   JOURNAL OF CLINICAL INVESTIGATION

ARFI and transient elastography for characterization of cystic fibrosis related liver disease: First longitudinal follow-up data in adult patients

(2013) Thomas Karlas et al.   Journal of Cystic Fibrosis

Gastrointestinal surgery in cystic fibrosis: A 20-year review

(2013) Paul J. Farrelly et al.   JOURNAL OF PEDIATRIC SURGERY

The role of impaired esophageal and gastric motility in end-stage lung diseases and after lung transplantation

(2013) Piero Marco Fisichella et al.   JOURNAL OF SURGICAL RESEARCH

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Allelic Variants Relate to Shifts in Faecal Microbiota of Cystic Fibrosis Patients

(2013) Serena Schippa et al.   PLoS One

Physiology of Epithelial Chloride and Fluid Secretion

(2013) R. A. Frizzell et al.   Cold Spring Harbor Perspectives in Medicine

Pancreatic Damage in Fetal and Newborn Cystic Fibrosis Pigs Involves the Activation of Inflammatory and Remodeling Pathways

(2012) Maisam Abu-El-Haija et al.   AMERICAN JOURNAL OF PATHOLOGY

Functional Cftr in crypt epithelium of organotypic enteroid cultures from murine small intestine

(2012) Jinghua Liu et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Ursodeoxycholate modulates bile flow and bile salt pool independently from the cystic fibrosis transmembrane regulator (Cftr) in mice

(2012) Frank A. J. A. Bodewes et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Pancreatic and biliary secretion are both altered in cystic fibrosis pigs

(2012) Aliye Uc et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Ileal smooth muscle dysfunction and remodeling in cystic fibrosis

(2012) P.-A. Risse et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Intestinal pH and Gastrointestinal Transit Profiles in Cystic Fibrosis Patients Measured by Wireless Motility Capsule

(2012) Daniel Gelfond et al.   DIGESTIVE DISEASES AND SCIENCES

Defects in Gallbladder Emptying and Bile Acid Homeostasis in Mice With Cystic Fibrosis Transmembrane Conductance Regulator Deficiencies

(2012) Dominique Debray et al.   GASTROENTEROLOGY

Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets

(2012) Alicia K. Olivier et al.   JOURNAL OF CLINICAL INVESTIGATION

Cirrhosis and other liver disease in cystic fibrosis

(2012) Thomas Flass et al.   Journal of Cystic Fibrosis

Absence of the cystic fibrosis transmembrane regulator (Cftr ) from myeloid-derived cells slows resolution of inflammation and infection

(2012) T. L. Bonfield et al.   JOURNAL OF LEUKOCYTE BIOLOGY

Intestinal Smooth Muscle Dysfunction Develops Postnatally in Cystic Fibrosis Mice

(2012) Robert C. De Lisle et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Cancer Risk in Cystic Fibrosis: A 20-Year Nationwide Study From the United States

(2012) Patrick Maisonneuve et al.   JNCI-Journal of the National Cancer Institute

Gastrointestinal transit times and motility in patients with cystic fibrosis

(2012) Caroline Hedsund et al.   SCANDINAVIAN JOURNAL OF GASTROENTEROLOGY

Variation in MSRA Modifies Risk of Neonatal Intestinal Obstruction in Cystic Fibrosis

(2012) Lindsay B. Henderson et al.   PLoS Genetics

Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium

(2011) Craig A. Hodges et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice

(2011) Romina Fiorotto et al.   GASTROENTEROLOGY

Mouse models of cystic fibrosis: Phenotypic analysis and research applications

(2011) Martina Wilke et al.   Journal of Cystic Fibrosis

Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease

(2011) Dominique Debray et al.   Journal of Cystic Fibrosis

An Activated Immune and Inflammatory Response Targets the Pancreas of Newborn Pigs with Cystic Fibrosis

(2011) Maisam Abu-El-Haija et al.   PANCREATOLOGY

Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice

(2011) L. S. Ostedgaard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The  F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs

(2011) L. S. Ostedgaard et al.   Science Translational Medicine

Pathology of Gastrointestinal Organs in a Porcine Model of Cystic Fibrosis

(2010) David K. Meyerholz et al.   AMERICAN JOURNAL OF PATHOLOGY

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

(2010) Jeng-Haur Chen et al.   CELL

Type of CFTR Mutation Determines Risk of Pancreatitis in Patients With Cystic Fibrosis

(2010) Chee Y. Ooi et al.   GASTROENTEROLOGY

Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy

(2010) Peter J. Lewindon et al.   HEPATOLOGY

The characterization of the first anti-mouse Muc6 antibody shows an increased expression of the mucin in pancreatic tissue of Cftr-knockout mice

(2010) Valérie Gouyer et al.   HISTOCHEMISTRY AND CELL BIOLOGY

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

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

Quantitative expression analyses of candidates for alternative anion conductance in cystic fibrosis mouse models

(2010) Josephine Braun et al.   Journal of Cystic Fibrosis

Small intestine bacterial overgrowth does not correspond to intestinal inflammation in cystic fibrosis

(2010) Aleksandra Lisowska et al.   SCANDINAVIAN JOURNAL OF CLINICAL & LABORATORY INVESTIGATION

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

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

The changing face of the exocrine pancreas in cystic fibrosis: the correlation between pancreatic status, pancreatitis and cystic fibrosis genotype

(2009) Arie Augarten et al.   EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY

Genetic Modifiers of Liver Disease in Cystic Fibrosis

(2009) Jaclyn R. Bartlett   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Small Intestinal Nonmeckelian Diverticulosis

(2009) Konstantinos Makris et al.   JOURNAL OF CLINICAL GASTROENTEROLOGY

Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator–dependent bicarbonate secretion

(2009) Mary Abigail S. Garcia et al.   JOURNAL OF CLINICAL INVESTIGATION

Constipation in pediatric Cystic Fibrosis patients: An underestimated medical condition

(2009) H.P.J. van der Doef et al.   Journal of Cystic Fibrosis

Defining DIOS and Constipation in Cystic Fibrosis With a Multicentre Study on the Incidence, Characteristics, and Treatment of DIOS

(2009) Roderick H Houwen et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Enteric circular muscle dysfunction in the cystic fibrosis mouse small intestine

(2009) r. c. de lisle et al.   NEUROGASTROENTEROLOGY AND MOTILITY

Ducts isolated from the pancreas of CFTR-null mice secrete fluid

(2009) Patricia Pascua et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Pathophysiological basis of liver disease in cystic fibrosis employing a ΔF508 mouse model

(2008) Folke Freudenberg et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Generation of a conditional null allele forCftrin mice

(2008) Craig A. Hodges et al.   GENESIS

Fibrogenesis in pediatric cholestatic liver disease: Role of taurocholate and hepatocyte-derived monocyte chemotaxis protein-1 in hepatic stellate cell recruitment

(2008) Grant A. Ramm et al.   HEPATOLOGY

Neonatal spontaneous colonic perforation due to cystic fibrosis

(2008) M. M. F. Siddiqui et al.   PEDIATRIC SURGERY INTERNATIONAL

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

(2008) C. S. Rogers et al.   SCIENCE