Severe pulmonary hypertension after the discovery of the familial primary pulmonary hypertension gene

R. M. Tuder, M. E. Yeager, M. Geraci, H. A. Golpon, N. F. Voelkel

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

The recent discoveries of the familial primary pulmonary hypertension gene and somatic mutations in key cell growth and cell death regulatory genes in primary pulmonary hypertension have added a new dimension to severe pulmonary hypertension research. These findings have already impacted on how the disease is viewed, and ultimately, how severe pulmonary hypertension is diagnosed and treated. However, this new information raises several fundamental questions related to the role of bone morphogenetic protein receptor signalling in the control of lung vascular cell function. Furthermore, additional genes and gene products may also be involved in the pathogenesis of the disease. The way severe pulmonary hypertension is viewed and studied is no the verge of shifting from a vasoconstrictive to a cell growth paradigm.

Original languageEnglish (US)
Pages (from-to)1065-1069
Number of pages5
JournalEuropean Respiratory Journal
Volume17
Issue number6
DOIs
StatePublished - 2001
Externally publishedYes

Fingerprint

Pulmonary Hypertension
Bone Morphogenetic Protein Receptors
Genes
Regulator Genes
Growth
Blood Vessels
Cell Death
Lung
Mutation
Research
Familial Primary Pulmonary Hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Severe pulmonary hypertension after the discovery of the familial primary pulmonary hypertension gene. / Tuder, R. M.; Yeager, M. E.; Geraci, M.; Golpon, H. A.; Voelkel, N. F.

In: European Respiratory Journal, Vol. 17, No. 6, 2001, p. 1065-1069.

Research output: Contribution to journalArticle

Tuder, R. M. ; Yeager, M. E. ; Geraci, M. ; Golpon, H. A. ; Voelkel, N. F. / Severe pulmonary hypertension after the discovery of the familial primary pulmonary hypertension gene. In: European Respiratory Journal. 2001 ; Vol. 17, No. 6. pp. 1065-1069.
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