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Table 2 Comparison of cystic fibrosis disease phenotypes between human and animal models

From: Pathophysiologic consequences following inhibition of a CFTR-dependent developmental cascade in the lung

HUMAN DISEASE PHENOTYPE

CFTR KNOCKOUT MOUSE MODEL PHENOTYPE

TIUKO CF RAT PHENOTYPE

LUNG FIBROSIS

FIBROSIS AND INFLAMMATION

PRESENT BY 100 DAYS OF AGE

MRP8/14 ELEVATED

DETECTED IN G551D MOUSE

INCREASED PRENATALLY

AIRWAY REACTIVITY

NOT DETECTED

INCREASED WITH AGE

CHRONIC INFLAMMATION

DETECTED IN CONGENIC MICE

PRESENT BY 100 DAYS OF AGE