Animal models of cystic fibrosis

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Abstract

Animal models of cystic fibrosis, in particular several different mutant mouse strains obtained by homologous recombination, have contributed considerably to our understanding of CF pathology. In this review, we describe and compare the main phenotypic features of these models. Recent and possible future developments in this field are discussed.

Keywords

Cystic fibrosis transmembrane conductance regulator
Genetics
Lung diseases
Microbiology
Pathology
Disease models
Animal
Intestines
Pathology
Mice
Genetics

Abbreviations

AML
amiloride
Bc
Burkholderia cepacia
BMT
bumetanide
Ca-ion
Ca2+-ionophore ionomycin
DIDS
4,4′-diisothiocyanatostilbene-2,2′-disulfonic acid
ENaC
epithelial sodium channel
FABP
fatty-acid-binding protein
FSK
forskolin
Hi
Haemophilus influenzae
iNOS
intracellular nitric oxide syntethase
KO
knockout
LPS
lipopolysaccharide
MCC
mucociliary clearance
Pa
Pseudomonas aeruginosa
PCL
periciliary liquid layer
RSV
respiratory syncytial virus
Sa
Staphylococcus aureus

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