411 J.X.-J. Yuan et al. (eds.), Textbook of Pulmonary Vascular Disease, DOI 10.1007/978-0-387-87429-6_27, © Springer Science+Business Media, LLC 2011 Abstract In this chapter, we use the term “plasminogen system” to designate the pathway that controls the conversion of plasminogen to plasmin and the proximal consequences of plasminogen activation. The “plasmino- gen system” is synonymous with the “fibrinolytic system,” a nomenclature that emphasizes the primary substrate of plasmin, fibrin, and function, and the outcome of fibrin degradation, fibrinolysis. However, evidence documents vital roles of components of the plasminogen system in physiological and pathological processes which cannot be attributed to and clearly extend beyond its role in clot dissolution. In particular, components of the plasminogen system can influence cell invasion into tissue, which is dependent on the capacity of plasmin to degrade various extracellular matrix (ECM) proteins, including fibronectins, thrombospondins, von Willebrand factor, and laminin, and to activate certain matrix metalloproteases, enzymes that degrade still other ECM constituents. Many components of the plasminogen system also interact with cells to generate intracellular signals that affect cell survival, proliferation, adhesion, and migration. Some of these roles are directly relevant to endothelial cell (EC) and smooth muscle cell (SMC) biology and it is in this context that we consider the interaction of the plasminogen system with these vascular cells. Why should a book that focuses on pulmonary vas- cular disease have a chapter dedicated to the interaction of the plasminogen system with the vessel wall? The answer is straightforward; there is compelling evidence linking the plasminogen system to pathological processes in the lung. Keywords Fibrinolysis฀ •฀ Thrombosis฀ •฀ Cell฀ adhesion฀ •฀Migration฀•฀Endothelial฀cell฀•฀Smooth฀muscle฀cell฀•฀Fibrin฀ •฀Fibronectin฀•฀Plasmin 1 Introduction In this chapter, we use the term “plasminogen system” to designate the pathway that controls the conversion of plasmi- nogen to plasmin and the proximal consequences of plasmi- nogen activation. The “plasminogen system” is synonymous with the “fibrinolytic system,” a nomenclature that empha- sizes the primary substrate of plasmin, fibrin, and function, and the outcome of fibrin degradation, fibrinolysis. However, evidence from enumerable in vitro studies and numerous studies conducted in mice deficient in various components documents vital roles of components of the plasminogen system in physiological and pathological processes which cannot be attributed to and clearly extend beyond its role in clot dissolution. In particular, components of the plasmino- gen system can influence cell invasion into tissue, which is dependent on the capacity of plasmin to degrade various extracellular matrix (ECM) proteins, including fibronectins, thrombospondins, von Willebrand factor, and laminin, and to activate certain matrix metalloproteases, enzymes that degrade still other ECM constituents. Many components of the plasminogen system also interact with cells to generate intracellular signals that affect cell survival, proliferation, adhesion, and migration. Some of these roles are directly rel- evant to endothelial cell (EC) and smooth muscle cell (SMC) biology and it is in this context that we consider the interac- tion of the plasminogen system with these vascular cells. Why should a book that focuses on pulmonary physiol- ogy have a chapter dedicated to the interaction of the plas- minogen system with the vessel wall? The answer is straightforward; there is compelling evidence linking the plasminogen system to pathological processes in the lung. In bleomycin-induced pulmonary fibrosis, deficiencies of plasminogen,฀urokinase฀plasminogen฀activator฀ (uPA),฀uPA฀ receptor฀(u-PAR),฀and฀tissue฀plasminogen฀activator฀(tPA)฀in฀ mice, all components of the plasminogen system, alter the pathogenic response substantially [1, 2]. Similarly, the response to lung infections and endotoxin-induced lung injury is markedly altered in mice deficient in certain com- ponents of the plasminogen system, and expression patterns R.฀Das฀(*) Department of Molecular Cardiology, Cleveland Clinic Foundation, 9500฀Euclid฀Avenue฀NB-50,฀Cleveland,฀OH฀44195,฀USA e-mail: dasr@ccf.org Chapter 27 Interaction of the Plasminogen System with the Vessel Wall Riku Das and Edward F. Plow