Active collagen synthesis by pulmonary arteries in human primary pulmonary hypertension

Am J Pathol. 1993 Jul;143(1):121-9.


Immunohistochemistry was performed on lung tissue obtained from patients with severe unexplained pulmonary hypertension using an antibody to the amino-terminal end of the procollagen type I propeptide. This antibody identifies newly synthesized alpha I(I) procollagen before cleavage of the amino-terminal propeptide following secretion and, therefore, can identify sites of active collagen deposition. Procollagen was detected in the media, media and neointima, or neointima alone of a large number of small muscular arteries from hypertensive lungs. Normal adult lungs were negative. Neointimal cells in remodeled small muscular arteries stained positively for alpha-smooth muscle actin and desmin consistent with a smooth muscle lineage. These data suggest smooth muscle-like cells in small muscular arteries are actively synthesizing collagen in patients with severe unexplained pulmonary hypertension.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Actins / immunology
  • Antibodies, Monoclonal
  • Collagen / biosynthesis*
  • Desmin / immunology
  • Humans
  • Hypertension, Pulmonary / metabolism*
  • Hypertension, Pulmonary / pathology
  • Immunohistochemistry
  • Muscle, Smooth, Vascular / cytology
  • Procollagen / immunology
  • Pulmonary Artery / chemistry
  • Pulmonary Artery / metabolism*
  • Pulmonary Artery / pathology
  • Staining and Labeling


  • Actins
  • Antibodies, Monoclonal
  • Desmin
  • Procollagen
  • Collagen