The vitronectin-binding function of PAI-1 exacerbates lung fibrosis in mice

Blood. 2011 Aug 25;118(8):2313-21. doi: 10.1182/blood-2010-12-324574. Epub 2011 Jul 6.

Abstract

Plasminogen activator inhibitor-1 (PAI-1) is increased in the lungs of patients with pulmonary fibrosis, and animal studies have shown that experimental manipulations of PAI-1 levels directly influence the extent of scarring that follows lung injury. PAI-1 has 2 known properties that could potentiate fibrosis, namely an antiprotease activity that inhibits the generation of plasmin, and a vitronectin-binding function that interferes with cell adhesion to this extracellular matrix protein. To determine the relative importance of each PAI-1 function in lung fibrogenesis, we administered mutant PAI-1 proteins that possessed either intact antiprotease or vitronectin-binding activity to bleomycin-injured mice genetically deficient in PAI-1. We found that the vitronectin-binding capacity of PAI-1 was the primary determinant required for its ability to exacerbate lung scarring induced by intratracheal bleomycin administration. The critical role of the vitronectin-binding function of PAI-1 in fibrosis was confirmed in the bleomycin model using mice genetically modified to express the mutant PAI-1 proteins. We conclude that the vitronectin-binding function of PAI-1 is necessary and sufficient in its ability to exacerbate fibrotic processes in the lung.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Bleomycin / administration & dosage
  • Bleomycin / toxicity
  • Bronchoalveolar Lavage Fluid / chemistry
  • Collagen / metabolism
  • Disease Models, Animal
  • Humans
  • Hydroxyproline / metabolism
  • Lung / drug effects
  • Lung / metabolism
  • Lung / pathology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Mice, Transgenic
  • Mutant Proteins / genetics
  • Mutant Proteins / metabolism
  • Plasminogen Activator Inhibitor 1 / genetics
  • Plasminogen Activator Inhibitor 1 / metabolism
  • Protein Binding
  • Pulmonary Fibrosis / etiology*
  • Pulmonary Fibrosis / metabolism*
  • Pulmonary Fibrosis / pathology
  • Recombinant Proteins / genetics
  • Recombinant Proteins / metabolism
  • Serpin E2 / blood
  • Serpin E2 / deficiency
  • Serpin E2 / genetics
  • Serpin E2 / metabolism*
  • Vitronectin / blood
  • Vitronectin / metabolism*

Substances

  • Mutant Proteins
  • Plasminogen Activator Inhibitor 1
  • Recombinant Proteins
  • SERPINE1 protein, human
  • Serpin E2
  • Serpine2 protein, mouse
  • Vitronectin
  • Bleomycin
  • Collagen
  • Hydroxyproline