The role of bronchial epithelial cells in the pathogenesis of COPD in Z-alpha-1 antitrypsin deficiency

BACKGROUND: Alpha-1 antitrypsin is the main inhibitor of neutrophil elastase in the lung. Although it is principally synthesized by hepatocytes, alpha-1 antitrypsin is also secreted by bronchial epithelial cells. Gene mutations can lead to alpha-1 antitrypsin deficiency, with the Z variant being the...

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Bibliografiske detaljer
Main Authors: Pini, Laura, Tiberio, Laura, Venkatesan, Narayanan, Bezzi, Michela, Corda, Luciano, Luisetti, Maurizio, Ferrarotti, Ilaria, Malerba, Mario, Lomas, David A, Janciauskiene, Sabina, Vizzardi, Enrico, Modina, Denise, Schiaffonati, Luisa, Tantucci, Claudio
Format: Artigo
Sprog:Inglês
Udgivet: BioMed Central 2014
Fag:
Research
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4177581/
https://ncbi.nlm.nih.gov/pubmed/25218041
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s12931-014-0112-3
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