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