Journal
EXPERT REVIEW OF RESPIRATORY MEDICINE
Volume 10, Issue 2, Pages 207-222Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1586/17476348.2016.1127759
Keywords
Alpha-1 antitrypsin; alpha-1 antitrypsin deficiency; chronic obstructive pulmonary disease; TNF-alpha; ADAM-17; neutrophils; inflammation; alpha-1 antitrypsin augmentation therapy
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Funding
- Alpha-1 Foundation (U.S.)
- Medical Research Charities Group/Health Research Board
- Programme for Research in Third-Level Institutes (PRTLI)
- European Alpha-1-antitrypsin Laurell's Training Award (eALTA)
- US Alpha-1 Foundation
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Alpha-1 antitrypsin (AAT) deficiency (AATD) has traditionally been thought of as a genetic disorder characterized by lung destruction and early emphysema in a low AAT, and high neutrophil elastase (NE) environment in the lungs of affected individuals. Recently, a growing body of evidence has emerged to support the hypothesis that tumor necrosis factor alpha (TNF-alpha) is essential in the pathogenesis of both genetic AATD and non-genetic chronic obstructive pulmonary disease (COPD). Reports have highlighted the importance of TNF-alpha driven immune cell dysfunction in the development of lung disease in AATD. The authors discuss the role of AAT as a key modulator of TNF-alpha signaling firstly in the setting of AATD and secondly in other conditions where AAT augmentation therapy has potential utility as a novel therapy.
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