Reduced proteolysis of surfactant protein A and changes of the bronchoalveolar lavage fluid proteome by inhaled alpha 1-protease inhibitor in cystic fibrosis

Electrophoresis. 2001 Jan;22(1):165-71. doi: 10.1002/1522-2683(200101)22:1<165::AID-ELPS165>3.0.CO;2-H.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Administration, Inhalation
  • Adolescent
  • Bronchoalveolar Lavage Fluid
  • Cystic Fibrosis / drug therapy
  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis / microbiology
  • Endopeptidases / metabolism
  • Female
  • Humans
  • Male
  • Proteolipids / metabolism*
  • Proteome / metabolism*
  • Pulmonary Surfactant-Associated Protein A
  • Pulmonary Surfactant-Associated Proteins
  • Pulmonary Surfactants / metabolism*
  • Solutions
  • alpha 1-Antitrypsin / administration & dosage
  • alpha 1-Antitrypsin / therapeutic use*


  • Proteolipids
  • Proteome
  • Pulmonary Surfactant-Associated Protein A
  • Pulmonary Surfactant-Associated Proteins
  • Pulmonary Surfactants
  • Solutions
  • alpha 1-Antitrypsin
  • Endopeptidases