Inactivation of urokinase-type plasminogen activator receptor (uPAR) gene induces dermal and pulmonary fibrosis and peripheral microvasculopathy in mice: a new model of experimental scleroderma?
Manetti, Mirko × Rosa, Irene Milia, Anna Franca Guiducci, Serena Carmeliet, Peter Ibba-Manneschi, Lidia Matucci-Cerinic, Marco #
Annals of the Rheumatic Diseases vol:73 issue:9 pages:1700-9
Urokinase-type plasminogen activator receptor (uPAR) is a key component of the fibrinolytic system involved in extracellular matrix remodelling and angiogenesis. The cleavage/inactivation of uPAR is a crucial step in fibroblast-to-myofibroblast transition and has been implicated in systemic sclerosis (SSc) microvasculopathy. In the present study, we investigated whether uPAR gene inactivation in mice could result in tissue fibrosis and peripheral microvasculopathy resembling human SSc.