Table 1 Airway phenotypes of CF animal models compared to humans
From: Airway disease phenotypes in animal models of cystic fibrosis
|  | Human | CFTR knockout/mutant mice | β-ENaC mouse | Rat | Ferret | Pig |
|---|---|---|---|---|---|---|
ASL HEIGHT IN LOWER AIRWAYS | Reduced [107] | NR in lower airways Reduced in nasal epithelium [15] | Reduced in bronchi | Reduced in trachea | NR | Normal in trachea of newborn pigs [101] |
MUCOCILIARY CLEARANCE IN LOWER AIRWAYS | Impaired [108] | Impaired in trachea of some strains [45] (has been contested [46]) | Impaired in trachea [70] | Normal in young rats (<  3 months) [4] Impaired in the trachea of older rats (>  3 months) [77, 80] | Impaired in trachea [86] | Impaired in trachea [102] |
REDUCED CHLORIDE SECRETION IN AIRWAYS | Present | Present in nasal epithelium [21] Absent in lower airways [25] | Absent [69] | Present in nasal and tracheal epithelium [4] | Present in tracheal epithelium | Present in nasal, tracheal and bronchial epithelium |
SODIUM HYPERABSORPTION IN AIRWAYS | Present (has been contested [109]) | Present in nasal epithelium Absent in lower airways [25] | Present in trachea [69] | Absent in nasal and tracheal epithelium [4] | Absent in trachea | Absent in nasal and tracheal epithelium [101] |
MUCUS OBSTRUCTION IN LOWER AIRWAYS | Mucus plugging [113] | Absent [15] | Mucus plugging [69] | Increased stored nasal mucus [4] and preliminary evidence of mucus plugging of submucosal glands in large airways [77, 80] | Mucus plugging [86] | Mucus plugging [93] |
LUNG INFECTION | Present [114] | Absent [21] | Present in neonates but reduces with age [72] | Absent [4] | Present [86] | Present [94] |
AIRWAY INFLAMMATION | Present [115] | Absent [21] | Present [69] | Absent [4] | Present [86] | Absent in newborn pigs but develops overtime [94] |