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Article

BMPR2 mutation status influences bronchial vascular changes in pulmonary arterial hypertension.

The European respiratory journal - 1 Dec 2016

Ghigna Maria-Rosa, Guignabert Christophe, Montani David, Girerd Barbara, Jaïs Xavier, Savale Laurent, Hervé Philippe, Thomas de Montpréville Vincent, Mercier Olaf, Sitbon Olivier, Soubrier Florent, Fadel Elie, Simonneau Gérald, Humbert Marc, Dorfmüller Peter

Abstract excerpt

The impact of bone morphogenetic protein receptor 2 (BMPR2) gene mutations on vascular remodelling in pulmonary arterial hypertension (PAH) is unknown. We sought to identify a histological profile of BMPR2 mutation carriers.Clinical data and lung histology from 44 PAH patients were subjected to systematic analysis and morphometry.Bronchial artery hypertrophy/dilatation and bronchial angiogenesis, as well as...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 27811071
DOI: 10.1183/13993003.00464-2016

Genes

BMPR2
PAH

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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