alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage.

Abstract

Introduction: a,-antitrypsin and the serpins The maintenance of health requires a balance between the defensive and offensive systems ofthe body. One such balance that is now just becoming understood at the molecular level is the one between the enzymes that break down protein and the antiproteases that act as their inhibitors. Of particular importance is the group of closely related enzymes, the serine proteases, which are responsible for triggering the inflammatory cascades of the plasma such as coagulation, kinin release, fibrinolysis, and complement activation. The triggering by these enzymes of the plasma cascades is controlled by an appropriately specialized group of plasma protease inhibitors, e.g., antithrombin, antiplasmin, and the first component ofcomplement (C1)' inhibitor.

Proteomic Analysis of Peritoneal Dialysate Fluid in Patients with Different Types of Peritoneal Membranes

“…17 Monocytes can also produce R1-antitrypsin and may be the other source of R1-antitrypsin in the PDE. 17 R1-Antitrypsin can increase by 4-fold in the plasma of patients with acute-phase states.…”

Polymerization and Oxidation of Alpha-1-Antitrypsin in Pathogenesis of Emphysema

“…The main physiological role of A1AT is protection of lower respiratory tract by inhibiting proteases released from triggered neutrophiles including neutrophil elastase, cathepsin G, and proteinase-3 (Carrell, 1986). The target proteases of A1AT derive from azurophilic granules of polymorphonuclear neutrophils which participate in lysosomal bacterial digestion and neutrophil migration through the extracellular matrix at the sites of inflammation.…”

Diverse Activities for Proteinases in the Pathogenesis of Chronic Obstructive Pulmonary Disease

“…Serine proteinase inhibitors (Serpins) in plasma and interstitial fluids include 1 -anti-trypsin (AAT), 1 -antichymotrypsin, plasminogen activator inhibitors, 2 -plasmin inhibitor, and the universal inhibitor, 2 -macroglobulin ( 2 -M), which inhibits all four classes of enzymes (16). Secretory leukocyte proteinase inhibitor (SLPI) and elafin are inhibitors synthesized locally in the respiratory tract by epithelial cells.…”