BMPR2 encodes bone morphogenetic protein receptor type 2 (1,038 aa). On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Highest expression in Lung (45.1 TPM) and Artery Aorta (35.3 TPM).

Pulmonary hypertension, primary, 1 is associated with mutations in the BMPR2 gene on chromosome 2.

The BMPR2 protein participates in Signaling by BMP pathway.

BMPR2 is classified as a druggable target (Cell Surface, Druggable Genome, Kinase, and Serine Threonine Kinase categories) with score 8.7.

Genetic testing for BMPR2 is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for pulmonary hypertension, primary, 1 has been reported in the published literature.

Phenotype severity distribution: 2 always present features, 4 very common features, 5 common features.

No clinical trials have been registered for pulmonary hypertension, primary, 1.

152 publications have been identified in PubMed for pulmonary hypertension, primary, 1. Research spans Basic Science / Preclinical (31%), Review / Meta-Analysis (28%), and Epidemiology / Natural History (15%).

Recent Publications

• Lin Y (2026). [PMID: 41799188](https://pubmed.ncbi.nlm.nih.gov/41799188/). *Theranostics*. [Basic Science / Preclinical]

• Uygur B (2026). [PMID: 40982059](https://pubmed.ncbi.nlm.nih.gov/40982059/). *Herz*. [Diagnostic / Biomarker]

• Torun EG (2026). [PMID: 40715799](https://pubmed.ncbi.nlm.nih.gov/40715799/). *Pediatr Cardiol*. [Diagnostic / Biomarker]

• Shaukat M (2026). [PMID: 41611798](https://pubmed.ncbi.nlm.nih.gov/41611798/). *Sci Rep*. [Epidemiology / Natural History]

• Soliman YMA (2026). [PMID: 41865030](https://pubmed.ncbi.nlm.nih.gov/41865030/). *Sci Rep*. [Epidemiology / Natural History]

• Jutant EM (2026). [PMID: 41713948](https://pubmed.ncbi.nlm.nih.gov/41713948/). *Eur Respir J*. [Review / Meta-Analysis]

• Huang AP (2026). [PMID: 41352442](https://pubmed.ncbi.nlm.nih.gov/41352442/). *Cardiovasc Pathol*. [Review / Meta-Analysis]

• Gangavelli A (2026). [PMID: 41766785](https://pubmed.ncbi.nlm.nih.gov/41766785/). *US Cardiol*. [Review / Meta-Analysis]

• Bravo-Marqués R (2026). [PMID: 41758914](https://pubmed.ncbi.nlm.nih.gov/41758914/). *Rheumatology (Oxford)*. [Epidemiology / Natural History]

• Zarrabian B (2026). [PMID: 41412359](https://pubmed.ncbi.nlm.nih.gov/41412359/). *J Heart Lung Transplant*. [Epidemiology / Natural History]