Reduced proteolysis of surfactant protein A and changes of the bronchoalveolar lavage fluid proteome by inhaled α1-protease inhibitor in cystic fibrosis
✍ Scribed by Matthias Griese; Christina von Bredow; Peter Birrer
- Publisher
- John Wiley and Sons
- Year
- 2001
- Tongue
- English
- Weight
- 565 KB
- Volume
- 22
- Category
- Article
- ISSN
- 0173-0835
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✦ Synopsis
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 alpha 1-protease inhibitor (alpha 1-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 alpha 1-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 < 20 kDa were reduced compared to pretreatment values. Degradation products of SP-A were shown by immunoblotting, being reduced after alpha 1-PI treatment. This pilot study demonstrates that inhalation of alpha 1-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.