Elastase and the pathobiology of unexplained pulmonary hypertension

Chest. 1998 Sep;114(3 Suppl):213S-224S. doi: 10.1378/chest.114.3_supplement.213s.


Our laboratory has focused on the increased activity of an endogenous vascular elastase in the pathobiology of pulmonary hypertension and on the mechanisms by which it is upregulated and by which it orchestrates abnormal remodeling of the vessel wall, specifically the induction of growth factors, the induction of the glycoprotein tenascin, which amplifies the proliferative response, and fibronectin, which is critical to the process of smooth muscle migration in the context of neointimal formation. We explore strategies by which targetting these processes might arrest progression or induce regression of pulmonary vascular disease associated with unexplained pulmonary hypertension.

Publication types

  • Review

MeSH terms

  • Animals
  • Cell Division / physiology
  • Cell Movement / physiology
  • Fibromuscular Dysplasia / pathology
  • Fibromuscular Dysplasia / physiopathology
  • Fibronectins / physiology
  • Growth Substances / physiology
  • Humans
  • Hypertension, Pulmonary / pathology
  • Hypertension, Pulmonary / physiopathology*
  • Muscle, Smooth, Vascular / pathology
  • Muscle, Smooth, Vascular / physiopathology
  • Pancreatic Elastase / physiology*
  • Pulmonary Artery / pathology
  • Pulmonary Artery / physiopathology
  • Tenascin / physiology
  • Up-Regulation / physiology


  • Fibronectins
  • Growth Substances
  • Tenascin
  • Pancreatic Elastase