Article
α1-Antitrypsin Polymerizes in Alveolar Macrophages of Smokers With and Without α1-Antitrypsin Deficiency.
Chest - 1 Sept 2018
Bazzan Erica, Tinè Mariaenrica, Biondini Davide, Benetti Riccardo, Baraldo Simonetta, Turato Graziella, Fagiuoli Stefano, Sonzogni Aurelio, Rigobello Chiara, Rea Federico, Calabrese Fiorella, Foschino-Barbaro Maria Pia, Miranda Elena, Lomas David A, Saetta Marina, Cosio Manuel G
Abstract excerpt
BACKGROUND: The deficiency of α1-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...
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