Plasminogen mediates the pathological effects of urokinase-type plasminogen activator overexpression

Am J Pathol. 2004 Jun;164(6):2299-304. doi: 10.1016/S0002-9440(10)63786-8.

Abstract

Increased expression of urokinase-type plasminogen activator (uPA) and its receptor (uPAR) is associated with different pathological conditions. Both uPAR-mediated signaling and plasmin-catalyzed extracellular proteolysis may contribute to pathogenesis. To evaluate the involvement of plasminogen in such circumstances, we have taken advantage of transgenic mouse models in which overexpression of uPA and/or uPAR in enamel epithelium, basal epidermis, and hair follicles leads to a pathological phenotype; uPA transgenic mice have chalky-white incisors and, when uPAR is co-expressed, develop extensive alopecia, epidermal thickening, and subepidermal blisters. We report here that when these transgenic mice were backcrossed into a plasminogen-deficient (Plg-/-) background, the dental and skin phenotypes appeared completely normal. Heterozygous Plg+/- transgenic mice exhibited a haplo-insufficiency, with an intermediate or normal phenotype. These results do not argue in favor of a role for uPAR-mediated signaling in our experimental model; rather, they demonstrate an essential, dose-dependent, requirement for plasminogen in uPA-mediated tissue alterations. They also support the hypothesis that plasminogen could play a part in certain skin diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Crosses, Genetic
  • Dental Enamel / pathology
  • Dental Enamel Hypoplasia / genetics
  • Dental Enamel Hypoplasia / pathology
  • Gene Expression Regulation / drug effects*
  • Incisor / pathology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred CBA
  • Mice, Transgenic
  • Phenotype
  • Plasminogen / deficiency
  • Plasminogen / genetics*
  • Plasminogen / physiology*
  • Receptors, Cell Surface / genetics
  • Receptors, Cell Surface / metabolism
  • Receptors, Urokinase Plasminogen Activator
  • Tooth Abnormalities / genetics
  • Tooth Abnormalities / pathology
  • Urokinase-Type Plasminogen Activator / drug effects
  • Urokinase-Type Plasminogen Activator / genetics*

Substances

  • Plaur protein, mouse
  • Receptors, Cell Surface
  • Receptors, Urokinase Plasminogen Activator
  • Plasminogen
  • Urokinase-Type Plasminogen Activator