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Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic

Carl Atkinson1, Susan Stewart, Paul D Upton

  • 1Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital, Cambridge, UK.

Circulation
|April 10, 2002
PubMed
Abstract

Insights

Reduced bone morphogenetic protein type II receptor (BMPR-II) expression in the lungs is linked to pulmonary hypertension. This finding suggests BMPR-II plays a role in the vascular lesions characteristic of this severe disease.

Area of Science:

  • Cardiovascular Research
  • Pulmonary Medicine
  • Molecular Biology

Background:

  • Mutations in bone morphogenetic protein type II receptor (BMPR-II), a transforming growth factor-beta (TGF-beta) superfamily member, are implicated in primary pulmonary hypertension (PPH).
  • The cellular expression sites of BMPR-II in healthy and diseased lungs remain largely uncharacterized.

Purpose of the Study:

  • To investigate the cellular localization of BMPR-II in normal and pulmonary hypertensive lungs.
  • To correlate BMPR-II expression levels with the presence of BMPR2 mutations and disease severity.

Main Methods:

  • Immunohistochemistry was used to examine BMPR-II expression in lung tissue from patients with PPH, secondary pulmonary hypertension, and healthy donors.
  • In situ hybridization was performed to detect BMPR-II mRNA.
  • Patients were genotyped for BMPR2 mutations.

Main Results:

  • BMPR-II expression was primarily observed in vascular endothelium in normal lungs.
  • In pulmonary hypertension, BMPR-II staining varied but included endothelial and myofibroblast cells.
  • A significant reduction in BMPR-II expression was noted in the peripheral lung of PPH patients, particularly those with BMPR2 mutations.

Conclusions:

  • The cellular distribution of BMPR-II supports its role in the development of pulmonary vascular lesions in PPH.
  • Diminished BMPR-II expression may contribute to the vascular obliteration seen in severe pulmonary hypertension.

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