Animal models in the pathophysiology of cystic fibrosis

A Semaniakou, RP Croll, V Chappe - Frontiers in pharmacology, 2019 - frontiersin.org
Our understanding of the multiorgan pathology of cystic fibrosis (CF) has improved
impressively during the last decades, but we still lack a full comprehension of the disease …

New animal models of cystic fibrosis: what are they teaching us?

NW Keiser, JF Engelhardt - Current opinion in pulmonary …, 2011 - journals.lww.com
Basic and clinical cystic fibrosis research will benefit greatly from the comparative
pathophysiology of cystic fibrosis mice, pigs, and ferrets. Both similarities and differences …

[HTML][HTML] Development of a porcine model of cystic fibrosis

MJ Welsh, CS Rogers, DA Stoltz… - Transactions of the …, 2009 - ncbi.nlm.nih.gov
Cystic Fibrosis (CF) is a common autosomal recessive disease that affects multiple organs.
The lack of an animal model with manifestations like those typically found in humans has …

Cystic fibrosis mouse models

C Guilbault, Z Saeed, GP Downey… - American journal of …, 2007 - atsjournals.org
Animal models of cystic fibrosis (CF) are powerful tools that enable the study of the
mechanisms and complexities of human disease. Murine models have several intrinsic …

Comparative biology of cystic fibrosis animal models

JT Fisher, Y Zhang, JF Engelhardt - … and Protocols, Volume II: Methods and …, 2011 - Springer
Animal models of human diseases are critical for dissecting mechanisms of pathophysiology
and developing therapies. In the context of cystic fibrosis (CF), mouse models have been the …

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

AK Olivier, KN Gibson-Corley… - American Journal of …, 2015 - journals.physiology.org
Multiple organ systems, including the gastrointestinal tract, pancreas, and hepatobiliary
systems, are affected by cystic fibrosis (CF). Many of these changes begin early in life and …

Animal models of cystic fibrosis pathology: phenotypic parallels and divergences

GM Lavelle, MM White, N Browne… - BioMed research …, 2016 - Wiley Online Library
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased …

Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model

PR Durie, G Kent, MJ Phillips, CA Ackerley - The American journal of …, 2004 - Elsevier
The lack of an appropriate animal model with multiorgan pathology characteristic of the
human form of cystic fibrosis has hampered our understanding of the pathobiology of the …

Mouse models of cystic fibrosis

DJ Davidson, M Rolfe - TRENDS in Genetics, 2001 - cell.com
The development of mouse models for cystic fibrosis has provided the opportunity to dissect
disease pathogenesis, correlate genotype and phenotype, study disease-modifying genes …

[HTML][HTML] Animal and cell culture models for cystic fibrosis: which model is right for your application?

A McCarron, D Parsons, M Donnelley - The American journal of pathology, 2021 - Elsevier
Over the past 30 years, a range of cystic fibrosis (CF) animal models have been generated
for research purposes. Different species, including mice, rats, ferrets, rabbits, pigs, sheep …