Surfactant protein A and other bronchoalveolar lavage fluid proteins are altered in cystic fibrosis

Inflammation and proteolytic processes play an important role in the progression of cystic fibrosis (CF) lung disease, The goal of this study was to describe bronchoalveolar lavage fluid (BALF) protein pattern of CF patients in comparison to controls and to assess if there is proteolytic degradation of surfactant protein A (SP-A), an important innate host defence component of the lungs. BALFs from 17 clinically stable CF patients and from eight healthy children mere separated by two-dimensional gel electrophoresis, Silver staining,vas used to show BALF proteins and Western blotting to detect SP-A isoforms. In CF, BALF proteins of a low molecular weight less than or equal to 20 kD were more abundant than in controls, Various proteins were seen in CF which were not present in controls and rice versa. Degradation of SP-A was present in 15 of 17 CF BALFs but in none of the controls, in contrast polymeric isoforms mere seen in all controls and in four of 17 CF patients. Proteolytic damage to surfactant protein A and significant changes of normal bronchoalveolar lavage fluid proteins occur in lungs of cystic fibrosis patients, Identification of altered bronchoalveolar lavage fluid proteins may give new insights into pathogenic mechanisms and provide new targets for therapy.