α1-antitrypsin polymerizes in alveolar macrophages of smokers with and without α1-antitrypsin deficiency

E Bazzan, M Tinč, D Biondini, R Benetti, S Baraldo… - Chest, 2018 - Elsevier
Background The deficiency of α 1-antitrypsin (AAT) is secondary to misfolding and
polymerization of the abnormal Z-AAT in liver cells and is associated with lung emphysema.
Alveolar macrophages (AMs) produce AAT; however, it is not known whether Z-AAT can
polymerize in AMs, further decreasing lung AAT and promoting lung inflammation. Our
intention was to investigate whether AAT polymerizes in human AMs and to study the
possible relation between polymerization and degree of lung inflammation. Methods …