The activin A antagonist follistatin inhibits cystic fibrosis-like lung inflammation and pathology.

ABSTRACT: Cystic fibrosis (CF) is the most common life-limiting genetically acquired respiratory disorder. Patients with CF have thick mucus obstructing the airways leading to recurrent infections, bronchiectasis and neutrophilic airway inflammation culminating in deteriorating lung function. Current management targets airway infection and mucus clearance, but despite recent advances in care, life expectancy is still only 40 years. We investigated whether activin A is elevated in CF lung disease and whether inhibiting activin A with its natural antagonist follistatin retards lung disease progression. We measured serum activin A levels, lung function and nutritional status in CF patients. We studied the effect of activin A on CF lung pathogenesis by treating newborn CF transgenic mice (β-ENaC) intran

SUBMITTER: Hardy CL 

PROVIDER: S-EPMC4495664 | biostudies-literature | 2015 Jul

REPOSITORIES: biostudies-literature