The current concepts of the pathogenesis of emphysema hold that progressive, chronic destruction of the alveolar structures occurs because there was in imbalance between the proteases and antiproteases in the lower respiratory tract. In this context, proteases, particularly neutrophil elastase, work unimpeded to destroy the alveolar structures. This concept has evolved from consideration of pat...

The cover image is based on the Article High-level production of wild-type and oxidation-resistant recombinant alpha-1-antitrypsin in glycoengineered CHO cells by Izel Koyuturk et al., https://doi.org/10.1002/bit.28129. Image Credit: Yves Durocher.

We have observed an electrophoretically abnormal, nonfunctional species of alpha 1-antitrypsin in serum from patients who were receiving bone-marrow transplants for treatment of leukemia or aplastic anemia. Three of four patients in whose serum this protein appeared died soon after; the fourth recovered, and the disappearance of the abnormal alpha 1-antitrypsin paralleled his recovery. This sug...

Alpha-l-antitrypsin-Pittsburgh is a human variant that resulted from a point mutation in the plasma protease inhibitor, a,-antitrypsin (358 Met Arg). This defect in the a,-antitrypsin molecule causes it to have greatly diminished anti-elastase activity but markedly increased antithrombin activity. In this report, we demonstrate that this variant protein also has greatly increased inhibitory act...

BACKGROUND/AIM Alpha-1 antitrypsin deficiency may be a potential predisposing factor for interstitial lung fibrosis. We investigated alpha-1 antitrypsin levels and its polymorphisms in patients with interstitial lung disease. MATERIALS AND METHODS A total of 103 interstitial lung disease patients were compared. RESULTS The mean alpha-1 antitrypsin level in idiopathic interstitial pneumonia ...

A biologically active neutral peptide mediator is cleaved from a plasma protein substrate by an alpha-1-antitrypsin-inhibitable serine protease apparently residing on the membrane of the human neutrophil. The peptide mediator has an approximate mol wt of 1,000, and is distinguished from the kinin peptides by a neutral isoelectric point, susceptibility to inactivation by trypsin as well as chymo...