Reduced proteolysis of surfactant protein A and changes of the bronchoalveolar lavage fluid proteome by inhaled α1-protease inhibitor in cystic fibrosis

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Abstract
In cystic fibrosis (CF), the chronic neutrophilic inflammation of the airways results in proteolytic degradation of lung tissue early in the course of the disease. Inhalation of α1‐protease inhibitor (α1‐PI) may restore the protease‐antiprotease imbalance and thus lead to less tissue damage. To monitor its impacts on bronchoalveolar lavage (BAL) fluid protein pattern (proteome) and on surfactant protein A (SP‐A), eight young adults with CF inhaled 100 mg of α1‐PI twice daily over eight weeks. BAL fluids were obtained before and after inhalation. Total protein, the number and amount of proteins with a molecular mass 1‐PI treatment. This pilot study demonstrates that inhalation of α1‐PI is associated with biochemical changes consistent with reduced proteolysis. The display of the BAL proteome by two‐dimensional electrophoresis may be helpful to quantify the overall molecular changes associated with proteolytic or other lung injuries and offers the possibility to monitor directly therapeutic interventions.