The Role of Neutrophils in Alpha-1 Antitrypsin Deficiency.

  title={The Role of Neutrophils in Alpha-1 Antitrypsin Deficiency.},
  author={Cormac McCarthy and Emer P Reeves and Noel Gerry McElvaney},
  journal={Annals of the American Thoracic Society},
  volume={13 Suppl 4},
Alpha-1 antitrypsin deficiency (AATD) is characterized by low levels of circulating alpha-1 antitrypsin and an increased risk for emphysema, liver disease, and panniculitis. The reduced levels of alpha-1 antitrypsin in AATD predispose the lung to unopposed proteolytic activity, predominantly from neutrophil-derived proteases, chiefly neutrophil elastase. This leads to emphysema. The mechanisms subtending the liver disease are less well understood, but are probably due to a "gain-of function… CONTINUE READING
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