Neutrophil Fates in Bronchiectasis and Alpha-1 Antitrypsin Deficiency.
The neutrophil is a powerful cellular defender of the vulnerable interface between the environment and pulmonary tissues. This cell's potent weapons are carefully calibrated in the healthy state to maximize effectiveness in fighting pathogens while minimizing tissue damage and allowing for repair of what damage does occur. The three related chronic airway disorders of cystic fibrosis, non-cystic fibrosis bronchiectasis, and alpha-1 antitrypsin deficiency all demonstrate significant derangements of this homeostatic system that result in their respective pathologies. An important shared feature among them is the inefficient resolution of chronic inflammation that serves as a central means for neutrophil-driven lung damage resulting in disease progression. Examining the commonalities and divergences between these diseases in the light of their immunopathology is informative and may help guide us toward future therapeutics designed to modulate the neutrophil's interplay with the pulmonary environment.
1 Department of Medicine.|3 Program in Protease and Matrix Biology, University of Alabama at Birmingham, and.
Derek W
DW
Russell
1 Department of Medicine.|2 Gregory Fleming James Cystic Fibrosis Research Center, and.|3 Program in Protease and Matrix Biology, University of Alabama at Birmingham, and.|4 Medical Service, Birmingham Veterans Affairs Medical Center, Birmingham, Alabama.
Amit
A
Gaggar
1 Department of Medicine.|2 Gregory Fleming James Cystic Fibrosis Research Center, and.
George M
GM
Solomon
D001987:Bronchiectasis / Q000276:immunology*; D003550:Cystic Fibrosis / Q000276:immunology*; D018450:Disease Progression; D006801:Humans; D008168:Lung / Q000276:immunology*; D009504:Neutrophils / Q000276:immunology*; D019896:alpha 1-Antitrypsin Deficiency / Q000276:immunology*
alpha-1 antitrypsin deficiency; bronchiectasis; cystic fibrosis; neutrophil; neutrophil elastase