Long-term clinical outcomes following treatment with alpha 1-proteinase inhibitor for COPD associated with alpha-1 antitrypsin deficiency: a look at the evidence

Abstract Alpha-1 antitrypsin deficiency (AATD) is a common hereditary disorder caused by mutations in the SERPINA1 gene, which encodes alpha-1 antitrypsin (AAT; also known as alpha 1-proteinase inhibitor, A1-PI). An important function of A1-PI in the lung is to inhibit neutrophil elastase, one of va...

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Bibliographic Details
Main Authors: Franck F. Rahaghi, Marc Miravitlles
Format: Article
Language:English
Published: BMC 2017-05-01
Series:Respiratory Research
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12931-017-0574-1