BMPR2

Summary

BMPR2 (bone morphogenetic protein receptor type 2, HGNC:1078) is a protein-coding gene on chromosome 2q33.1-q33.2, encoding Bone morphogenetic protein receptor type-2 (Q13873). On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. It is haploinsufficient (ClinGen: sufficient evidence).

This gene encodes a member of the bone morphogenetic protein (BMP) receptor family of transmembrane serine/threonine kinases. The ligands of this receptor are members of the TGF-beta superfamily. BMPs are involved in endochondral bone formation and embryogenesis. These proteins transduce their signals through the formation of heteromeric complexes of two different types of serine (threonine) kinase receptors: type I receptors of about 50-55 kD and type II receptors of about 70-80 kD. Mutations in this gene have been associated with primary pulmonary hypertension, both familial and fenfluramine-associated, and with pulmonary venoocclusive disease.

Source: NCBI Gene 659 — RefSeq curated summary.

At a glance

• Gene–disease (curated): pulmonary arterial hypertension (Definitive, ClinGen) — +5 more curated relationships
• GWAS associations: 9
• Clinical variants (ClinVar): 2,167 total — 483 pathogenic, 82 likely-pathogenic
• Phenotypes (HPO): 23
• Druggable target: yes — 19 molecules with ChEMBL bioactivity
• Dosage sensitivity (ClinGen): haploinsufficiency sufficient evidence, triplosensitivity no evidence
• MANE Select transcript: NM_001204

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:1078
Approved symbol	BMPR2
Name	bone morphogenetic protein receptor type 2
Location	2q33.1-q33.2
Locus type	gene with protein product
Status	Approved
Aliases	BRK-3, T-ALK, BMPR3, BMPR-II
Ensembl gene	ENSG00000204217
Ensembl biotype	protein_coding
OMIM	600799
Entrez	659

Gene structure

Transcript identifiers

Ensembl transcripts: 3 — 2 protein_coding, 1 protein_coding_CDS_not_defined

ENST00000374574, ENST00000374580, ENST00000479069

RefSeq mRNA: 1 — MANE Select: NM_001204 NM_001204

CCDS: CCDS33361

Canonical transcript exons

ENST00000374580 — 13 exons

Exon	Start	End
ENSE00001463885	202555252	202556531
ENSE00001463886	202552716	202552888
ENSE00001463887	202542311	202542447
ENSE00001463889	202532585	202532732
ENSE00001463891	202530794	202530954
ENSE00001463892	202520087	202520201
ENSE00001463894	202518822	202519052
ENSE00001463895	202514888	202514979
ENSE00001463897	202513719	202513829
ENSE00002269353	202464809	202464979
ENSE00002273038	202376327	202377550
ENSE00003462485	202559696	202567749
ENSE00003530769	202467519	202467689

Expression profiles

Bgee: expression breadth ubiquitous, 271 present calls, max score 97.01.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 21.5807 / max 165.5949, expressed in 1787 samples.

FANTOM5 promoters (8 alternative TSS)

Promoter ID	TPM avg	Samples expressed
24719	13.0989	1749
24717	4.8391	1603
24718	1.1907	817
24721	0.9176	499
24720	0.5414	329
24723	0.4035	190
24716	0.3787	150
202537	0.2110	85

Top tissues by expression

293 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
visceral pleura	UBERON:0002401	97.01	gold quality
lower lobe of lung	UBERON:0008949	96.63	gold quality
tendon of biceps brachii	UBERON:0008188	96.59	gold quality
cortical plate	UBERON:0005343	95.51	gold quality
urethra	UBERON:0000057	95.50	gold quality
lateral nuclear group of thalamus	UBERON:0002736	95.07	gold quality
parietal pleura	UBERON:0002400	94.80	gold quality
right lung	UBERON:0002167	94.63	gold quality
upper leg skin	UBERON:0004262	94.60	gold quality
pleura	UBERON:0000977	94.55	gold quality
skin of hip	UBERON:0001554	94.51	gold quality
cauda epididymis	UBERON:0004360	94.43	gold quality
tibia	UBERON:0000979	94.40	gold quality
heart right ventricle	UBERON:0002080	94.06	gold quality
ventricular zone	UBERON:0003053	93.94	gold quality
pons	UBERON:0000988	93.75	gold quality
middle temporal gyrus	UBERON:0002771	93.74	gold quality
dorsal root ganglion	UBERON:0000044	93.71	gold quality
ganglionic eminence	UBERON:0004023	93.67	gold quality
caput epididymis	UBERON:0004358	93.35	gold quality
medial globus pallidus	UBERON:0002477	93.28	gold quality
adrenal tissue	UBERON:0018303	93.27	gold quality
tendon	UBERON:0000043	93.24	gold quality
corpus epididymis	UBERON:0004359	93.08	gold quality
seminal vesicle	UBERON:0000998	93.07	gold quality
superior vestibular nucleus	UBERON:0007227	93.05	gold quality
postcentral gyrus	UBERON:0002581	92.69	gold quality
adult organism	UBERON:0007023	92.55	gold quality
synovial joint	UBERON:0002217	92.52	gold quality
parietal lobe	UBERON:0001872	92.46	gold quality

Single-cell (SCXA)

Detected in 8 experiment(s), a significant marker in 6.

Experiment	Marker?	Max mean expression
E-GEOD-135922	yes	42.18
E-CURD-119	yes	25.16
E-MTAB-9543	yes	8.81
E-MTAB-9067	yes	6.34
E-GEOD-130148	yes	5.28
E-CURD-112	no	3.12
E-MTAB-6379	no	0.94
E-ANND-3	no	0.00

Regulation

Is transcription factor: yes

Downstream targets (CollecTRI)

3 targets.

Target	Regulation
HAMP	Activation
POSTN	Activation
RUNX2	Activation

Upstream regulators (CollecTRI, top): ACVRL1, ESR1, GDF2, SMAD3, SP3, STAT3, TBXT

miRNA regulators (miRDB)

420 targeting BMPR2, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-5692A	100.00	74.40	6850
HSA-MIR-3163	100.00	77.23	8605
HSA-MIR-3646	100.00	73.56	5283
HSA-MIR-1252-5P	100.00	69.80	2774
HSA-LET-7A-3P	100.00	74.03	3932
HSA-LET-7B-3P	100.00	74.08	3913
HSA-LET-7F-1-3P	100.00	74.02	3928
HSA-MIR-98-3P	100.00	74.08	3907
HSA-MIR-190A-3P	100.00	80.35	5520
HSA-MIR-3613-3P	100.00	76.36	7965
HSA-MIR-5011-5P	100.00	83.46	5820
HSA-MIR-8485	100.00	77.57	4731
HSA-MIR-4768-5P	100.00	69.49	2861
HSA-MIR-4747-5P	100.00	67.90	2681
HSA-MIR-5196-5P	100.00	67.98	2761
HSA-MIR-6873-3P	100.00	71.42	2626
HSA-MIR-6833-3P	100.00	70.63	3197
HSA-MIR-4533	100.00	69.48	2758
HSA-MIR-340-5P	100.00	72.50	4437
HSA-MIR-4682	100.00	68.89	1258
HSA-MIR-450A-1-3P	100.00	69.33	1837
HSA-MIR-548AW	99.99	72.57	3559
HSA-MIR-4282	99.99	75.36	6408
HSA-MIR-548C-3P	99.99	74.01	7587
HSA-MIR-3662	99.99	73.82	5684
HSA-MIR-6077	99.99	68.04	2299
HSA-MIR-3667-3P	99.99	67.17	1636
HSA-MIR-371B-5P	99.99	75.34	4759
HSA-MIR-4789-3P	99.99	70.75	2484
HSA-MIR-3617-3P	99.98	67.86	918

Functional genomics

ClinGen dosage: haploinsufficiency 3 (sufficient evidence), triplosensitivity 0 (no evidence). ClinGen Gene Dosage Map

Literature-anchored findings (GeneRIF, showing 40)

• signals induced by binding of BMP-2 to preformed receptor complexes activate the Smad pathway, whereas BMP-2-induced recruitment of receptors activates a different, Smad-independent pathway (PMID:11714695)
• Bone morphogenetic protein receptor type II is a receptor for growth differentiation factor-9 (PMID:12135884)
• BMPRII has a role in signal transduction and its mutation can lead to primary pulmonary hopertension (PMID:12221115)
• bone morphogenetic protein receptor 2 mutations that appear to be rare in the general population but may combine with exposure to fenfluramine derivatives to greatly increase the risk of developing severe pulmonary arterial hypertension (PMID:12358323)
• Overexpression of a dominant negative type II bone morphogenetic protein receptor inhibits the growth of human breast cancer cells. (PMID:12543773)
• Decreased expression of bone morphogenetic protein (BMP) receptor type II correlates with insensitivity to BMP-6 in human renal cell carcinoma cells (PMID:14676131)
• Loss of BMPRII signaling in mouse smooth muscle (due to dominant negative human transgene)is sufficient to produce the pulmonary hypertensive phenotype. (PMID:15031260)
• Mutations of BMPR2 underlie many cases of familial primary pulmonary hypertension. (PMID:15055271)
• results indicate that a substantial portion of Japanese primary pulmonary hypertension patients carry BMPR2 mutations with considerable heterogeneity (PMID:15146475)
• Pulmonary hypertension in children may have a different genetic background than in adults and we postulate a recessive mode of inheritance in a proportion of infantile cases. (PMID:15295086)
• Loss of BMP-RII function may result in increased tumorigenicity in human prostate cancer cells. (PMID:15354178)
• Human bladder TCC tissues have a frequent loss of BMP-RII expression and that overexpression of BMP-RII leads to restoration of BMP signaling and decreased tumor growth in the human bladder TCC cell line TSU-Pr1. (PMID:15492256)
• Mutations may promote expansion of fibroblasts resistant to antiproliferative, prodifferentiation effects of bone morphogenetic proteins. Possible mechanism for vascular obliteration in familial pulmonary hypertension. (PMID:15516492)
• frequency of BMPR2 mutations in a different population of patients with sporadic idiopathic pulmonary arterial hypertension (PMID:15591269)
• 2 novel mutations were found in BMPR2 from 18 patients developing PAH before 6 years of age, including one partial gene deletion and one nonsense mutation. (PMID:15687131)
• Somatic loss of wild-type BMPR2 alleles is unlikely to play a significant role in the pathogenesis of familial pulmonary arterial hypertension. (PMID:15699281)
• BMPR2 gene rearrangements is associated with primary pulmonary hypertension (PMID:15775752)
• BMPR2 mutations have a role in short lifetime expectancy in primary pulmonary hypertension (PMID:15965979)
• c-Src tyrosine kinase was identified as a binding partner of the BMPR-II C-terminus. (PMID:16002577)
• Bone morphogenetic protein-2 is the predominant family member expressed in NSCLC and is overexpressed in the majority of human lung carcinomas independent of cell type. (PMID:16122479)
• Our data suggest that the complex formation between c-kit and BMPR-II leads to phosphorylation of BMPR-II at Ser757, which modulates BMPR-II-dependent signaling (PMID:16155937)
• Bone morphogenetic protein regulaates K(V) channel expression and that loss of this signaling pathway in pulmonary artery through a mutation in BMPR2. (PMID:16339782)
• The correlation of the SERT promoter polymorphism with age at diagnosis in FPAH suggests a possible relationship between the SERT and BMPR2. (PMID:16339917)
• TGF-beta type II receptor BMPR2 is mutated in pulmonary arterial hypertension (PMID:16429395)
• BMPR2 gene rearrangements have a role in mutations in familial and idiopathic pulmonary arterial hypertension (PMID:16429403)
• Human granulosa-like tumor cell line KGN expressed BMP type I (BMPR1A and BMPR1B) and type II receptors (BMPR2) and the BMP signaling molecules SMADs (SMAD1 and SMAD5). (PMID:16436528)
• Finally, two inhibitors of cyclin-dependent kinase 9 (a dominant negative CDK9 and flavopiridol) repressed activity from the MR and BMPR2 promoters (PMID:16615932)
• Patients with familial or idiopathic PAH and nonsynonymous BMPR2 variations are unlikely to demonstrate vasoreactivity (PMID:16717148)
• Novel signaling network relevant to pulmonary artery hypertension underscored by BMPR2 mutations. (PMID:16782755)
• Characterization of this enzyme expressed in E coli cells. (PMID:16982201)
• Atrial septal defect Eisenmenger syndrome may occur without BMPR2 mutation. (PMID:17102831)
• HGF up-regulates the expression of the bone morphogenetic protein receptors, BMPR-IB and BMPR-II, in prostate cancer cells (PMID:17203235)
• This protein, in transgenic rats, provide a rationale for the development of gene therapy aimed at improving BMPR2 signaling. (PMID:17277049)
• Germline mutations in the gene coding for the bone morphogenetic protein receptor II (BMPR2) are present in more than 70% of FPAH and up to 26% of idiopathic, apparently sporadic cases (IPAH). (PMID:17318011)
• BMP pathway regulates IL-6 in pulmonary tissues and conversely that IL-6 regulates the BMP pathway (PMID:17322283)
• negligible change in the emission maximum was observed when bis-ANS binds ecBMPR-II. FRET revealed close proximity between tryptophan residue & bis-ANS binding site. (PMID:17363346)
• RGMa facilitates the use of ActRIIA by endogenous BMP2 and BMP4 ligands that otherwise prefer signaling via BMPRII and that increased utilization of ActRIIA leads to generation of an enhanced BMP signal (PMID:17472960)
• primary pulmonary arterial smooth muscle cells demonstrated colocalization of BMPRII and RACK1 in vivo. RACK1-BMPRII interaction was reduced when kinase domain mutations occurring in patients with PAH were introduced to BMPRII (PMID:17515463)
• Trb3 is a critical component of a novel mechanism for regulation of the bone morphogenetic protein pathway by BMPRII. (PMID:17576816)
• Expression of BMP-4 and BMP-7 and their receptors in human ovaries from fetuses as well as adults. (PMID:17624341)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	bmpr2a	ENSDARG00000011941
danio_rerio	bmpr2b	ENSDARG00000020057
mus_musculus	Bmpr2	ENSMUSG00000067336
rattus_norvegicus	Bmpr2	ENSRNOG00000022196

Paralogs (11): TGFBR1 (ENSG00000106799), BMPR1A (ENSG00000107779), ACVR2B (ENSG00000114739), ACVR1 (ENSG00000115170), ACVR2A (ENSG00000121989), ACVR1C (ENSG00000123612), AMHR2 (ENSG00000135409), ACVR1B (ENSG00000135503), BMPR1B (ENSG00000138696), ACVRL1 (ENSG00000139567), TGFBR2 (ENSG00000163513)

Protein

Protein identifiers

Bone morphogenetic protein receptor type-2 — Q13873 (reviewed: Q13873)

Alternative names: Bone morphogenetic protein receptor type II

All UniProt accessions (1): Q13873

UniProt curated annotations — full annotation on UniProt →

Function. On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Can also mediate signaling through the activation of the p38MAPK cascade. Binds to BMP7, BMP2 and, less efficiently, BMP4. Binding is weak but enhanced by the presence of type I receptors for BMPs. Mediates induction of adipogenesis by GDF6. Promotes signaling also by binding to activin A/INHBA.

Subunit / interactions. Interacts with GDF5. Interacts with BMP4. Interacts with SCUBE3. Interacts with TSC22D1/TSC-22. Interacts with activin A/INHBA.

Subcellular location. Cell membrane.

Tissue specificity. Highly expressed in heart and liver.

Disease relevance. Pulmonary hypertension, primary, 1 (PPH1) [MIM:178600] A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial pulmonary hypertension is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs. The disease is caused by variants affecting the gene represented in this entry. Pulmonary venoocclusive disease 1, autosomal dominant (PVOD1) [MIM:265450] A disease characterized by widespread fibrous obstruction and intimal thickening of septal veins and preseptal venules, a low diffusing capacity for carbon monoxide, occult alveolar hemorrhage, and nodular ground-glass opacities, septal lines and lymph node enlargement showed by high-resolution computed tomography of the chest. It is frequently associated with pulmonary capillary dilatation and proliferation, and is a rare and devastating cause of pulmonary hypertension. The disease is caused by variants affecting the gene represented in this entry.

Similarity. Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.

Isoforms (2)

UniProt ID	Names	Canonical?
Q13873-1	1	yes
Q13873-2	2

RefSeq proteins (1): NP_001195* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR000333	TGFB_receptor	Family
IPR000472	Activin_recp	Domain
IPR000719	Prot_kinase_dom	Domain
IPR011009	Kinase-like_dom_sf	Homologous_superfamily
IPR017441	Protein_kinase_ATP_BS	Binding_site
IPR045860	Snake_toxin-like_sf	Homologous_superfamily

Pfam: PF00069, PF01064

Catalyzed reactions (Rhea), 2 shown:

• L-seryl-[receptor-protein] + ATP = O-phospho-L-seryl-[receptor-protein] + ADP + H(+) (RHEA:18673)
• L-threonyl-[receptor-protein] + ATP = O-phospho-L-threonyl-[receptor-protein] + ADP + H(+) (RHEA:44880)

UniProt features (103 total): sequence variant 34, helix 18, strand 14, binding site 5, disulfide bond 5, modified residue 4, turn 4, compositionally biased region 3, glycosylation site 3, region of interest 3, topological domain 2, splice variant 2, signal peptide 1, chain 1, active site 1, transmembrane region 1, domain 1, sequence conflict 1

Structure

Experimental structures (PDB)

7 structures.

PDB	Method	Resolution (Å)
2HLQ	X-RAY DIFFRACTION	1.45
7PPA	X-RAY DIFFRACTION	1.48
6UNP	X-RAY DIFFRACTION	2.3
3G2F	X-RAY DIFFRACTION	2.35
7PPB	X-RAY DIFFRACTION	2.4
7U5O	X-RAY DIFFRACTION	3.45
7PPC	X-RAY DIFFRACTION	3.6

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q13873-F1	58.07	0.27

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Catalytic / active sites (1): 333 (proton acceptor)

Ligand- & substrate-binding residues (5): 209–217; 230; 280–282; 337–338; 351

Post-translational modifications (4): 379, 586, 680, 681

Disulfide bonds (5): 34–66, 60–84, 94–117, 99–116, 118–123

Glycosylation sites (3): 55, 110, 126

Function

Pathways and Gene Ontology

Reactome pathways

3 pathways

ID	Pathway
R-HSA-201451	Signaling by BMP
R-HSA-162582	Signal Transduction
R-HSA-9006936	Signaling by TGFB family members

MSigDB gene sets: 697 (showing top): GSE45365_NK_CELL_VS_CD8_TCELL_UP, GOBP_CARDIAC_CHAMBER_DEVELOPMENT, GOBP_SMAD_PROTEIN_SIGNAL_TRANSDUCTION, RNGTGGGC_UNKNOWN, GCM_MAP4K4, GOBP_EMBRYO_DEVELOPMENT_ENDING_IN_BIRTH_OR_EGG_HATCHING, AAGCAAT_MIR137, GOBP_EPITHELIUM_DEVELOPMENT, GOBP_MUSCLE_TISSUE_DEVELOPMENT, GOBP_CARDIAC_SEPTUM_DEVELOPMENT, GGTGTGT_MIR329, MYOGENIN_Q6, GOBP_OUTFLOW_TRACT_SEPTUM_MORPHOGENESIS, GOBP_REGULATION_OF_BLOOD_PRESSURE, GOBP_VENTRICULAR_SEPTUM_MORPHOGENESIS

GO Biological Process (69): MAPK cascade (GO:0000165), angiogenesis (GO:0001525), blood vessel development (GO:0001568), osteoblast differentiation (GO:0001649), mesoderm formation (GO:0001707), cell fate specification (GO:0001708), maternal placenta development (GO:0001893), endothelial cell proliferation (GO:0001935), lymphangiogenesis (GO:0001946), blood vessel remodeling (GO:0001974), chondrocyte development (GO:0002063), negative regulation of systemic arterial blood pressure (GO:0003085), outflow tract morphogenesis (GO:0003151), aortic valve development (GO:0003176), pulmonary valve development (GO:0003177), mitral valve morphogenesis (GO:0003183), tricuspid valve morphogenesis (GO:0003186), endocardial cushion development (GO:0003197), obsolete negative regulation of cell proliferation involved in heart valve morphogenesis (GO:0003252), protein import into nucleus (GO:0006606), cell surface receptor protein serine/threonine kinase signaling pathway (GO:0007178), cellular response to starvation (GO:0009267), anterior/posterior pattern specification (GO:0009952), positive regulation of gene expression (GO:0010628), positive regulation of epithelial cell migration (GO:0010634), regulation of lung blood pressure (GO:0014916), cell differentiation (GO:0030154), proteoglycan biosynthetic process (GO:0030166), negative regulation of cell growth (GO:0030308), positive regulation of bone mineralization (GO:0030501), BMP signaling pathway (GO:0030509), regulation of cell population proliferation (GO:0042127), positive regulation of osteoblast differentiation (GO:0045669), positive regulation of ossification (GO:0045778), negative regulation of vasoconstriction (GO:0045906), positive regulation of transcription by RNA polymerase II (GO:0045944), lung alveolus development (GO:0048286), negative regulation of smooth muscle cell proliferation (GO:0048662), positive regulation of axon extension involved in axon guidance (GO:0048842), stem cell differentiation (GO:0048863)

GO Molecular Function (17): transforming growth factor beta receptor activity (GO:0005024), ATP binding (GO:0005524), activin receptor activity, type II (GO:0016362), growth factor binding (GO:0019838), BMP binding (GO:0036122), cadherin binding (GO:0045296), metal ion binding (GO:0046872), BMP receptor activity (GO:0098821), protein tyrosine kinase binding (GO:1990782), nucleotide binding (GO:0000166), protein kinase activity (GO:0004672), protein serine/threonine kinase activity (GO:0004674), transmembrane receptor protein serine/threonine kinase activity (GO:0004675), protein binding (GO:0005515), kinase activity (GO:0016301), transferase activity (GO:0016740), signaling receptor activity (GO:0038023)

GO Cellular Component (18): obsolete extracellular space (GO:0005615), nucleoplasm (GO:0005654), plasma membrane (GO:0005886), caveola (GO:0005901), clathrin-coated pit (GO:0005905), adherens junction (GO:0005912), basal plasma membrane (GO:0009925), cell surface (GO:0009986), postsynaptic density (GO:0014069), apical plasma membrane (GO:0016324), axon (GO:0030424), dendrite (GO:0030425), neuronal cell body (GO:0043025), signaling receptor complex (GO:0043235), membrane (GO:0016020), cell junction (GO:0030054), organelle (GO:0043226), cell body (GO:0044297)

Reactome top-level categories

Rollup of top-2 pathways:

Category	Pathways
Signaling by TGFB family members	1
Signal Transduction	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cellular anatomical structure	6
anatomical structure formation involved in morphogenesis	2
anatomical structure development	2
anatomical structure morphogenesis	2
semi-lunar valve development	2
atrioventricular valve morphogenesis	2
transmembrane receptor protein serine/threonine kinase activity	2
membrane	2
plasma membrane region	2
neuron projection	2
intracellular signaling cassette	1
blood vessel morphogenesis	1
vasculature development	1
ossification	1
cell differentiation	1
formation of primary germ layer	1
mesoderm morphogenesis	1
cell fate commitment	1
cellular developmental process	1
placenta development	1
developmental process involved in reproduction	1
maternal process involved in female pregnancy	1
epithelial cell proliferation	1
lymph vessel morphogenesis	1
tissue remodeling	1
chondrocyte differentiation	1
cell development	1
regulation of systemic arterial blood pressure	1
negative regulation of blood pressure	1
heart morphogenesis	1
mitral valve development	1
tricuspid valve development	1
heart development	1
mesenchyme development	1
intracellular protein transport	1
protein localization to nucleus	1
import into nucleus	1
establishment of protein localization to organelle	1
adenyl ribonucleotide binding	1
purine ribonucleoside triphosphate binding	1

Protein interactions and networks

STRING

2946 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
BMPR2	BMP4	P12644	998
BMPR2	BMP7	P18075	997
BMPR2	GDF2	Q9UK05	997
BMPR2	BMP2	P12643	996
BMPR2	BMP6	P22004	989
BMPR2	BMPR1A	P36894	986
BMPR2	ACVR1	Q04771	986
BMPR2	BMPR1B	P78366	985
BMPR2	GDF9	O60383	977
BMPR2	ENG	P17813	971
BMPR2	BMP15	O95972	950
BMPR2	ACVRL1	P37023	938
BMPR2	DYNLT1	P63172	935
BMPR2	ACVR2A	P27037	928
BMPR2	ACVR2B	Q13705	923

IntAct

58 interactions, top by confidence:

A	B	Type	Score
CD83	BTAF1	psi-mi:“MI:0914”(association)	0.530
ACVR1	BMPR1A	psi-mi:“MI:0914”(association)	0.530
MRAP2	GOLIM4	psi-mi:“MI:0914”(association)	0.530
KCNE3	RIOK3	psi-mi:“MI:0914”(association)	0.530
BMPR2	PRKG1	psi-mi:“MI:0915”(physical association)	0.520
PRKG1	BMPR2	psi-mi:“MI:0915”(physical association)	0.520
BMPR2	Prkcb	psi-mi:“MI:0915”(physical association)	0.500
Prkcb	BMPR2	psi-mi:“MI:0915”(physical association)	0.500
BMPR2	C4bpa	psi-mi:“MI:0915”(physical association)	0.500
NRAS	ESYT2	psi-mi:“MI:2364”(proximity)	0.480
BMP2	BMPR2	psi-mi:“MI:0407”(direct interaction)	0.440
BMPR2	GDF5	psi-mi:“MI:0407”(direct interaction)	0.440
GDF5	BMPR2	psi-mi:“MI:0407”(direct interaction)	0.440
Fstl1	BMPR2	psi-mi:“MI:0915”(physical association)	0.400
BMPR2	YWHAE	psi-mi:“MI:0915”(physical association)	0.400
TGFBR1	BMPR2	psi-mi:“MI:0915”(physical association)	0.400
HSCB	BMPR2	psi-mi:“MI:0915”(physical association)	0.370
BMPR2	MAPK8	psi-mi:“MI:0915”(physical association)	0.370
PYCARD	MYO1C	psi-mi:“MI:0914”(association)	0.350
RUSF1	MAP1LC3B2	psi-mi:“MI:0914”(association)	0.350
MRAP2	GOSR1	psi-mi:“MI:0914”(association)	0.350
RNF11	SDCBP	psi-mi:“MI:0914”(association)	0.350
LRRIQ1	TNFRSF10B	psi-mi:“MI:0914”(association)	0.350
ARMH4	FGFR1	psi-mi:“MI:0914”(association)	0.350
NS3	C15orf61	psi-mi:“MI:0914”(association)	0.350

BioGRID (148): BMPR2 (Affinity Capture-MS), BMPR2 (Two-hybrid), BMPR2 (Proximity Label-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-MS), BMPR2 (Affinity Capture-RNA), CES3 (Affinity Capture-MS), GCGR (Affinity Capture-MS), Zfp788 (Affinity Capture-MS), Actr8 (Affinity Capture-MS)

ESM2 similar proteins: A0A0U1QT59, A2VEY9, A8X9H4, D3KZG3, O35412, O35607, O57474, O61366, O93383, P18861, P29415, P34535, P36383, P43322, P49414, P50605, P60571, P91682, Q02297, Q03345, Q05199, Q11069, Q13873, Q14693, Q2THW7, Q2THW9, Q2THX1, Q5R838, Q5RJX2, Q5YCC7, Q64448, Q69ZW3, Q6DR98, Q6H1V1, Q6IMP4, Q6TYA8, Q7TQ69, Q7Z5M5, Q86B91, Q8INR6

Diamond homologs: A0A0P0XII1, A0A0R0HPY5, A7J1T2, C0LGD6, C0LGD8, C0LGD9, C0LGG3, C0LGR6, C0LGV0, O00238, O00506, O04086, O35607, O46680, O64556, O65440, P04627, P09560, P0C5E2, P10398, P10533, P14056, P15056, P18161, P20792, P27037, P27038, P27039, P27040, P27041, P27966, P28028, P34908, P36894, P36895, P36896, P36897, P36898, P37023, P37172

SIGNOR signaling

24 interactions.

A	Effect	B	Mechanism
GDF6	up-regulates	BMPR2	binding
BMP15	up-regulates	BMPR2	binding
CTDNEP1	down-regulates	BMPR2	binding
SMURF1	down-regulates	BMPR2	ubiquitination
BMPR2	up-regulates	BMPR1A	phosphorylation
BMPR2	up-regulates	BMPR1B	phosphorylation
SMURF2	down-regulates	BMPR2	ubiquitination
NOG	“down-regulates activity”	BMPR2	binding
SMURF	down-regulates	BMPR2	ubiquitination
BMP7	up-regulates	BMPR2	binding
BMP4	up-regulates	BMPR2	binding
BMP2	up-regulates	BMPR2	binding
BMPR2	up-regulates	ACVR1	binding
BMPR2	“form complex”	ACVR1/BMPR2	binding
BMPR2	“form complex”	BMPR1A/1B/2	binding
BMPR2	up-regulates	BMPR1B	binding
BMPR2	“up-regulates activity”	BMPR1A	binding
BMPR1A	up-regulates	BMPR2	binding
BMP2	“up-regulates activity”	BMPR2	binding
BMPR2	“up-regulates activity”	BMPR1B	binding
BMPR2	up-regulates	BMPR1A	binding

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 69 IntAct physical interaction partners (hypergeometric vs the genome-wide background, BH-FDR, gene-set size 15–500, ranked by fold). A functional readout of the neighbourhood — distinct from this gene’s own memberships above, and biased toward well-studied / hub proteins, so read it as themes rather than proof.

Reactome pathways:

Pathway	Partners	Fold	FDR
NCAM signaling for neurite out-growth	6	38.8×	3e-06
RAF/MAP kinase cascade	5	7.3×	4e-03

GO biological processes:

GO term	Partners	Fold	FDR
positive regulation of SMAD protein signal transduction	5	33.6×	1e-04
MAPK cascade	8	21.5×	3e-06
Ras protein signal transduction	5	18.0×	2e-03
in utero embryonic development	6	7.6×	1e-02

Disease & clinical

Clinical variants and AI predictions

ClinVar

2167 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	483
Likely pathogenic	82
Uncertain significance	899
Likely benign	484
Benign	84

Top pathogenic / likely-pathogenic (30)

Variant ID	HGVS	Classification
1067745	NM_001204.7(BMPR2):c.1472G>T (p.Arg491Leu)	Pathogenic
1069537	NM_001204.7(BMPR2):c.2450_2451del (p.Asn817fs)	Pathogenic
1069660	NM_001204.7(BMPR2):c.1748dup (p.Asn583fs)	Pathogenic
1069902	NM_001204.7(BMPR2):c.897_903del (p.Ser300fs)	Pathogenic
1071169	NC_000002.11:g.(?203241529)(203242283_?)del	Pathogenic
1072447	NM_001204.7(BMPR2):c.610A>T (p.Lys204Ter)	Pathogenic
1073354	NM_001204.7(BMPR2):c.1206del (p.Lys402fs)	Pathogenic
1073527	NM_001204.7(BMPR2):c.1102G>T (p.Glu368Ter)	Pathogenic
1075069	NC_000002.11:g.(?203329512)(203332432_?)del	Pathogenic
1075070	NC_000002.11:g.(?203378432)(203379712_?)del	Pathogenic
1076573	NM_001204.7(BMPR2):c.524del (p.Met174_Leu175insTer)	Pathogenic
1195010	NM_001204.7(BMPR2):c.1091del (p.Val364fs)	Pathogenic
1256042	NM_001204.7(BMPR2):c.961_963dup (p.Arg321dup)	Pathogenic
1328388	NM_001204.7(BMPR2):c.530-2A>G	Pathogenic
1330302	NM_001204.7(BMPR2):c.1519_1520insAT (p.Ile507fs)	Pathogenic
1330492	NM_001204.7(BMPR2):c.369del (p.Leu122_Cys123insTer)	Pathogenic
1339409	NM_001204.7(BMPR2):c.1524G>A (p.Trp508Ter)	Pathogenic
1372691	NM_001204.7(BMPR2):c.894G>A (p.Trp298Ter)	Pathogenic
1379840	NM_001204.7(BMPR2):c.1069del (p.Arg357fs)	Pathogenic
1387800	NM_001204.7(BMPR2):c.120T>A (p.Tyr40Ter)	Pathogenic
1409534	NC_000002.11:g.(?203329522)(203379712_?)del	Pathogenic
1419785	NC_000002.11:g.(?203407024)(203407180_?)del	Pathogenic
1423038	NM_001204.7(BMPR2):c.409dup (p.Thr137fs)	Pathogenic
1424050	NM_001204.7(BMPR2):c.1332dup (p.Thr445fs)	Pathogenic
1443330	NM_001204.7(BMPR2):c.346del (p.Cys116fs)	Pathogenic
1452839	NM_001204.7(BMPR2):c.135del (p.Gly45_Ile46insTer)	Pathogenic
1453966	NM_001204.7(BMPR2):c.320C>A (p.Ser107Ter)	Pathogenic
145553	GRCh38/hg38 2q33.1-33.2(chr2:202445130-202631487)x1	Pathogenic
1456366	NM_001204.7(BMPR2):c.1175del (p.Val392fs)	Pathogenic
1459305	NM_001204.7(BMPR2):c.1028dup (p.Asn343fs)	Pathogenic

SpliceAI

3190 predictions. Top by Δscore:

Variant	Effect	Δscore
2:202464802:A:AG	acceptor_gain	1.0000
2:202464807:A:AG	acceptor_gain	1.0000
2:202464808:G:GT	acceptor_gain	1.0000
2:202464808:GC:G	acceptor_gain	1.0000
2:202464808:GCT:G	acceptor_gain	1.0000
2:202464808:GCTT:G	acceptor_gain	1.0000
2:202464808:GCTTC:G	acceptor_gain	1.0000
2:202467686:CTCA:C	donor_gain	1.0000
2:202467688:CAG:C	donor_loss	1.0000
2:202467689:AG:A	donor_loss	1.0000
2:202467690:G:GG	donor_gain	1.0000
2:202467690:GTA:G	donor_loss	1.0000
2:202467691:TAA:T	donor_loss	1.0000
2:202467692:AA:A	donor_loss	1.0000
2:202467694:G:GG	donor_gain	1.0000
2:202494545:G:GT	donor_gain	1.0000
2:202509135:G:GT	donor_gain	1.0000
2:202509157:GGGA:G	donor_gain	1.0000
2:202513713:TTTTA:T	acceptor_loss	1.0000
2:202513714:TTTA:T	acceptor_loss	1.0000
2:202513715:TTAG:T	acceptor_loss	1.0000
2:202513716:TAG:T	acceptor_loss	1.0000
2:202513717:A:AG	acceptor_gain	1.0000
2:202513717:A:AT	acceptor_loss	1.0000
2:202513718:G:GG	acceptor_gain	1.0000
2:202513826:ACAGG:A	donor_loss	1.0000
2:202513827:CAGG:C	donor_loss	1.0000
2:202513828:AG:A	donor_loss	1.0000
2:202513829:GGT:G	donor_loss	1.0000
2:202513830:GTAAA:G	donor_loss	1.0000

AlphaMissense

6824 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
2:202464938:T:C	L69P	1.000
2:202464940:T:A	W70R	1.000
2:202464940:T:C	W70R	1.000
2:202464942:G:C	W70C	1.000
2:202464942:G:T	W70C	1.000
2:202467526:G:C	W85C	1.000
2:202467526:G:T	W85C	1.000
2:202467566:T:A	C99S	1.000
2:202467566:T:C	C99R	1.000
2:202467567:G:C	C99S	1.000
2:202518858:G:C	G220R	1.000
2:202518880:T:A	V227D	1.000
2:202518883:C:A	A228D	1.000
2:202518890:A:C	K230N	1.000
2:202518890:A:T	K230N	1.000
2:202530821:G:C	R332P	1.000
2:202530824:A:C	D333A	1.000
2:202530824:A:T	D333V	1.000
2:202530878:A:C	D351A	1.000
2:202530878:A:G	D351G	1.000
2:202530878:A:T	D351V	1.000
2:202532588:G:C	G378R	1.000
2:202532589:G:A	G378D	1.000
2:202532619:T:C	L388P	1.000
2:202532624:G:A	G390R	1.000
2:202532624:G:C	G390R	1.000
2:202532669:G:C	D405H	1.000
2:202532669:G:T	D405Y	1.000
2:202532670:A:T	D405V	1.000
2:202542430:T:A	W466R	1.000

dbSNP variants (sampled 300 via entrez): RS1000004717 (2:202483468 G>A,T), RS1000015667 (2:202500084 C>T), RS1000057815 (2:202392053 C>G,T), RS1000058117 (2:202380598 A>G), RS1000058273 (2:202484466 A>C), RS1000077570 (2:202465784 C>T), RS1000094721 (2:202470625 G>A), RS1000108294 (2:202391820 G>A,C), RS1000108629 (2:202381368 G>A,C), RS1000116915 (2:202526521 T>C), RS1000119004 (2:202439801 G>A,C), RS1000136110 (2:202446228 T>G), RS1000136266 (2:202533840 A>G), RS1000153274 (2:202504959 G>A,C), RS1000163357 (2:202518487 C>T)

Disease associations

OMIM: gene MIM:600799 | disease phenotypes: MIM:178600, MIM:265450, MIM:106600, MIM:234810, MIM:135100

GenCC curated gene-disease

Disease	Classification	Inheritance
pulmonary hypertension, primary, 1	Definitive	Autosomal dominant
pulmonary venoocclusive disease 1	Strong	Autosomal dominant
heritable pulmonary arterial hypertension	Supportive	Autosomal dominant
primary ovarian failure	Limited	Autosomal dominant

ClinGen Gene-Disease Validity (2)

Expert-panel classifications — Definitive > Strong > Moderate > Limited > Disputed > Refuted.

Disease	Classification	Inheritance
congenital heart disease	Limited	AD
pulmonary arterial hypertension	Definitive	AD

Mondo (11): pulmonary hypertension, primary, 1 (MONDO:0024533), pulmonary venoocclusive disease 1 (MONDO:0020713), pulmonary arterial hypertension (MONDO:0015924), drug- or toxin-induced pulmonary arterial hypertension (MONDO:0017149), tooth agenesis, selective, 1 (MONDO:0007129), mitral valve prolapse (MONDO:0004910), pulmonary hypertension (MONDO:0005149), pulmonary venoocclusive disease 2 (MONDO:0009329), fibrodysplasia ossificans progressiva (MONDO:0007606), heritable pulmonary arterial hypertension (MONDO:0017148), primary ovarian failure (MONDO:0005387)

Orphanet (12): Idiopathic/heritable pulmonary arterial hypertension (Orphanet:422), Pulmonary venoocclusive disease (Orphanet:31837), Pulmonary arterial hypertension (Orphanet:182090), Rare genetic premature ovarian failure (Orphanet:485382), Drug- or toxin-induced pulmonary arterial hypertension (Orphanet:275786), Pulmonary arterial hypertension associated with another disease (Orphanet:275791), Pulmonary arterial hypertension associated with connective tissue disease (Orphanet:275798), Pulmonary arterial hypertension associated with congenital heart disease (Orphanet:275803), Pulmonary arterial hypertension associated with HIV infection (Orphanet:275808), Oligodontia (Orphanet:99798), Pulmonary capillary hemangiomatosis (Orphanet:199241), Fibrodysplasia ossificans progressiva (Orphanet:337)

HPO phenotypes

23 total (24 of 23 shown, HPO-id order):

HPO	Term
HP:0000006	Autosomal dominant inheritance
HP:0000822	Hypertension
HP:0001009	Telangiectasia
HP:0001667	Right ventricular hypertrophy
HP:0001708	Right ventricular failure
HP:0001977	Abnormal thrombosis
HP:0002092	Pulmonary arterial hypertension
HP:0002094	Dyspnea
HP:0003596	Middle age onset
HP:0003829	Typified by incomplete penetrance
HP:0004964	Pulmonary arterial medial hypertrophy
HP:0005168	Elevated right atrial pressure
HP:0005308	Pulmonary artery vasoconstriction
HP:0005312	Pulmonary aterial intimal fibrosis
HP:0005317	Increased pulmonary vascular resistance
HP:0006518	Pulmonary venous occlusion
HP:0011353	Arterial intimal fibrosis
HP:0011462	Young adult onset
HP:0012735	Cough
HP:0025180	Centrilobular ground-glass opacification on pulmonary HRCT
HP:0030848	Elevated jugular venous pressure
HP:0030879	Interlobular septal thickening
HP:0031687	Abnormally loud pulmonic component of the second heart sound
HP:0001634	Mitral valve prolapse

GWAS associations

9 associations (top):

Study	Trait	p-value
GCST001791_16	Urate levels	3.000000e-06
GCST005316_173	Intelligence (MTAG)	2.000000e-08
GCST007931_13	Medication use (HMG CoA reductase inhibitors)	2.000000e-08
GCST010173_93	Triglyceride levels	5.000000e-10
GCST010244_134	Triglyceride levels	6.000000e-13
GCST010320_37	PR interval	5.000000e-08
GCST90002386_272	High light scatter reticulocyte percentage of red cells	4.000000e-13
GCST90002390_464	Mean corpuscular hemoglobin	7.000000e-09
GCST90002406_65	Reticulocyte fraction of red cells	8.000000e-13

EFO canonical traits (6, from GWAS)

EFO ID	Trait name
EFO:0004531	urate measurement
EFO:0004337	intelligence
EFO:0009932	HMG CoA reductase inhibitor use measurement
EFO:0004530	triglyceride measurement
EFO:0004462	PR interval
EFO:0004527	mean corpuscular hemoglobin

MeSH disease descriptors (5)

Descriptor	Name	Tree numbers
D006976	Hypertension, Pulmonary	C08.381.423; C14.907.489.556
D008945	Mitral Valve Prolapse	C14.280.484.400.500
D016649	Primary Ovarian Insufficiency	C12.050.351.500.056.630.750; C12.100.250.056.630.750; C19.391.630.750
D000081029	Pulmonary Arterial Hypertension	C08.381.423.847
C535861	Hemangiomatosis, familial pulmonary capillary (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (1): CHEMBL5467 (SINGLE PROTEIN)

Molecules with ChEMBL bioactivity

19 molecules (phase ≥1), by development phase (incl. off-target/promiscuous compounds). Patent mentions across the top 20 by phase: 137,610 (via chembl_molecule»patent_compound — counts attach to the compound, not the gene–compound relationship, so off-target/promiscuous molecules can dominate).

Molecule	Name	Phase	Patents
CHEMBL1287853	FEDRATINIB	4	3,554
CHEMBL1789941	RUXOLITINIB	4	11,547
CHEMBL288441	BOSUTINIB	4	12,255
CHEMBL4435170	DEUCRAVACITINIB	4	679
CHEMBL502835	NINTEDANIB	4	8,545
CHEMBL535	SUNITINIB	4	79,020
CHEMBL223360	LINIFANIB	3	3,925
CHEMBL274654	ORANTINIB	3	3,596
CHEMBL522892	DOVITINIB	3	4,944
CHEMBL603469	LESTAURTINIB	3
CHEMBL1230165	SILMITASERTIB	2	593
CHEMBL1721885	SU-014813	2	363
CHEMBL3120215	OSI-027	2	1,854
CHEMBL495727	AT-9283	2	1,376
CHEMBL572878	TOZASERTIB	2	2,998
CHEMBL1908397	KW-2449	1	622
CHEMBL3545083	RGB-286638	1	551
CHEMBL4289017	PF-03814735	1	537
CHEMBL482967	CYC-116	1	651

PharmGKB: 1 entry (VIP=true, CPIC=false)

GtoPdb / IUPHAR curated pharmacology

(IUPHAR/BPS Guide to Pharmacology — expert-curated)

Target class: catalytic receptor — Type II receptor serine/threonine kinases

Most potent curated ligand interactions (2 total), top 2:

Ligand	Action	Affinity	Parameter
compound 13a [PMID: 23639540]	Inhibition	7.8	pIC50
deucravacitinib	Inhibition	6.71	pIC50

Binding affinities (BindingDB)

6 measured of 6 human assays (6 total across all organisms); most potent 6 below. Values come from heterogeneous assays and are not directly comparable.

Ligand	Measure	Value
1-[4-(3-amino-1H-indazol-4-yl)phenyl]-3-(2-fluoro-5-methyl-phenyl)urea	KD	450 nM
(3Z)-4-amino-5-fluoro-3-[5-(4-methylpiperazino)-1,3-dihydrobenzimidazol-2-ylidene]carbostyril	KD	520 nM
N-[4-({4-[(3-methyl-1H-pyrazol-5-yl)amino]-6-(4-methylpiperazin-1-yl)pyrimidin-2-yl}sulfanyl)phenyl]cyclopropanecarboxamide	KD	1100 nM
5-[(Z)-(5-fluoranyl-2-oxidanylidene-1H-indol-3-ylidene)methyl]-2,4-dimethyl-N-[(2S)-3-morpholin-4-yl-2-oxidanyl-propyl]-1H-pyrrole-3-carboxamide	KD	2600 nM
1-Acyl-1H-[1,2,4]triazole-3,5-diamine Analogue 3b	KD	3100 nM
N-[2-(diethylamino)ethyl]-5-[(Z)-(5-fluoro-2-oxo-1,2-dihydro-3H-indol-3-ylidene)methyl]-2,4-dimethyl-1H-pyrrole-3-carboxamide	KD	3500 nM

ChEMBL bioactivities

91 potent at pChembl≥5 of 92 total, top 50 by pChembl (potency: 10 = 0.1 nM, 6 = 1 µM).

pChembl	Type	Value	Unit	Molecule
8.52	Kd	3	nM	CHEMBL400402
8.30	Ki	5	nM	CHEMBL5404016
8.21	Ki	6.2	nM	CHEMBL5404269
8.15	Ki	7	nM	CHEMBL5422418
8.10	Kd	8	nM	PF-03814735
8.10	Ki	8	nM	CHEMBL5434353
7.83	Kd	14.69	nM	CHEMBL5653589
7.80	IC50	15.9	nM	CHEMBL2385579
7.80	Ki	16	nM	CHEMBL5420022
7.76	ED50	17.45	nM	CHEMBL5653589
7.75	Ki	18	nM	CHEMBL5437902
7.72	Kd	19	nM	ORANTINIB
7.69	Ki	20.6	nM	CHEMBL5398151
7.66	Ki	22	nM	CHEMBL5436042
7.62	IC50	24	nM	CHEMBL2385600
7.44	IC50	36.2	nM	CHEMBL1235645
7.41	Ki	39	nM	CHEMBL5428614
7.40	Ki	40	nM	CHEMBL5402879
7.37	Kd	43	nM	AT-9283
7.25	Kd	56	nM	NINTEDANIB
7.23	IC50	59	nM	CHEMBL2385586
7.13	IC50	74	nM	DORSOMORPHIN
7.08	Kd	83.5	nM	CHEMBL5271697
7.06	Ki	88	nM	CHEMBL5421520
7.05	Kd	89	nM	CHEMBL1241674
6.93	Ki	118	nM	CHEMBL5409160
6.82	Kd	153	nM	RGB-286638
6.73	Kd	186	nM	CHEMBL1235645
6.71	IC50	193	nM	DEUCRAVACITINIB
6.70	Kd	200	nM	SU-014813
6.66	Ki	220	nM	CHEMBL5408480
6.66	Ki	220	nM	CHEMBL5397482
6.63	Ki	236	nM	CHEMBL5399442
6.60	Ki	250	nM	CHEMBL5400260
6.51	Kd	310	nM	CHEMBL1908842
6.51	Ki	310	nM	CHEMBL5438139
6.51	Kd	310	nM	CHEMBL1908395
6.43	Kd	370	nM	KW-2449
6.37	Ki	430	nM	CHEMBL5411422
6.34	IC50	461	nM	CHEMBL5271220
6.31	IC50	488	nM	CHEMBL5079149
6.30	IC50	506	nM	CHEMBL5271697
6.24	Kd	570	nM	SUNITINIB
6.24	Kd	580	nM	BOSUTINIB
6.20	IC50	630	nM	CHEMBL5278418
6.17	Kd	675	nM	SU-014813
6.16	IC50	695	nM	CHEMBL2385591
6.06	Kd	880	nM	JNJ-7706621
6.03	Ki	930	nM	CHEMBL5434246
6.03	Kd	930	nM	RUXOLITINIB

PubChem BioAssay actives

85 with measured affinity, of 550 total; 50 most potent distinct compounds. Largely complementary to BindingDB; screening values are coarse (µM, 4 dp), so sub-nM hits tie at the floor.

Compound	Assay	Type	Value	Unit
4-chloro-3-[5-methyl-3-[4-(2-pyrrolidin-1-ylethoxy)anilino]-1,2,4-benzotriazin-7-yl]phenol	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	0.0030	uM
3-[[5-ethoxy-4-(3-oxo-4-phenylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0050	uM
N-[(3-carbamoylphenyl)methyl]-2-isoquinolin-6-yl-3-[(1R,3R)-3-(methylcarbamoyl)cyclopentyl]benzimidazole-5-carboxamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0062	uM
3-[[5-methoxy-4-(3-oxo-4-phenylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0070	uM
N-[(3-carbamoylphenyl)methyl]-3-cyclopentyl-2-isoquinolin-6-ylbenzimidazole-5-carboxamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0080	uM
N-[2-[4-[[4-(cyclobutylamino)-5-(trifluoromethyl)pyrimidin-2-yl]amino]-11-azatricyclo[6.2.1.02,7]undeca-2(7),3,5-trien-11-yl]-2-oxoethyl]acetamide	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	0.0080	uM
4-methyl-3-[(2-methyl-6-pyridin-3-ylpyrazolo[3,4-d]pyrimidin-4-yl)amino]-N-[3-(trifluoromethyl)phenyl]benzamide	2147951: Binding affinity to human BMPR2 incubated for 45 mins by Kinobead based pull down assay	kd	0.0147	uM
4-[4-[3-(1H-pyrazol-4-yl)pyrazolo[1,5-a]pyrimidin-6-yl]phenyl]morpholine	750126: Inhibition of BMPR2 (unknown origin)	ic50	0.0159	uM
3-[[5-methoxy-4-(4-pyridin-2-ylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0160	uM
3-[[4-(3-fluoro-3-phenylazetidin-1-yl)-5-methoxypyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0180	uM
3-[2,4-dimethyl-5-[(Z)-(2-oxo-1H-indol-3-ylidene)methyl]-1H-pyrrol-3-yl]propanoic acid	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	0.0190	uM
N-[[3-[4-[5-methoxy-2-(3-sulfamoylanilino)pyrimidin-4-yl]piperazin-1-yl]phenyl]methyl]-4-[3-(methylamino)-3-oxopropyl]benzamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0206	uM
N-[(3-carbamoylphenyl)methyl]-2-isoquinolin-6-yl-3-methylbenzimidazole-5-carboxamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0220	uM
6-(4-methoxyphenyl)-3-(1H-pyrazol-4-yl)pyrazolo[1,5-a]pyrimidine	750126: Inhibition of BMPR2 (unknown origin)	ic50	0.0240	uM
2-[4-[[4-[(5-cyclopropyl-1H-pyrazol-3-yl)amino]pyrimidin-2-yl]amino]phenyl]acetonitrile	1948605: Inhibition of BMPR2 (174 to end residues) (unknown origin) using MBP as substrate incubated for 60 mins in presence of ATP by ADP Glo assay	ic50	0.0362	uM
N-benzyl-2-isoquinolin-6-yl-3-[(1R,3R)-3-(methylcarbamoyl)cyclopentyl]benzimidazole-5-carboxamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0390	uM
3-[[5-methoxy-4-(4-phenylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0400	uM
1-cyclopropyl-3-[5-[6-(morpholin-4-ylmethyl)-1H-benzimidazol-2-yl]-1H-pyrazol-4-yl]urea	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	0.0430	uM
methyl 2-hydroxy-3-[N-[4-[methyl-[2-(4-methylpiperazin-1-yl)acetyl]amino]phenyl]-C-phenylcarbonimidoyl]-1H-indole-6-carboxylate	1948593: Inhibition of BMPR2 (unknown origin) assessed as dissociation constant	kd	0.0560	uM
6-(4-piperazin-1-ylphenyl)-3-(1H-pyrazol-4-yl)pyrazolo[1,5-a]pyrimidine	750126: Inhibition of BMPR2 (unknown origin)	ic50	0.0590	uM
6-[4-(2-piperidin-1-ylethoxy)phenyl]-3-pyridin-4-ylpyrazolo[1,5-a]pyrimidine	1948594: Inhibition of BMPR2 (unknown origin)	ic50	0.0740	uM
2,4,8,10,11,14,23-heptazatetracyclo[15.2.2.13,7.19,12]tricosa-1(19),3,5,7(23),9,12(22),17,20-octaen-13-one	1948602: Inhibition of BMPR2 (unknown origin) assessed as dissociation constant by ITC assay	kd	0.0835	uM
3-[[5-methoxy-4-(2-methyl-4-phenylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.0880	uM
2-(4-amino-1-propan-2-ylpyrazolo[3,4-d]pyrimidin-3-yl)-1H-indol-5-ol	624826: Binding constant for BMPR2 kinase domain	kd	0.0890	uM
3-[[5-methoxy-4-(3-methyl-4-phenylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.1180	uM
1-[3-[4-[[4-(2-methoxyethyl)piperazin-1-yl]methyl]phenyl]-4-oxo-1H-indeno[2,1-d]pyrazol-5-yl]-3-morpholin-4-ylurea	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	0.1530	uM
Deucravacitinib	1579046: Inhibition of BMPR2 (unknown origin)	ic50	0.1930	uM
5-[(Z)-(5-fluoro-2-oxo-1H-indol-3-ylidene)methyl]-N-[(2S)-2-hydroxy-3-morpholin-4-ylpropyl]-2,4-dimethyl-1H-pyrrole-3-carboxamide	435780: Binding constant for BMPR2 kinase domain	kd	0.2000	uM
3-[[5-methoxy-4-(8-phenyl-3,8-diazabicyclo[3.2.1]octan-3-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.2200	uM
3-[[5-methoxy-4-(7-phenyl-4,7-diazaspiro[2.5]octan-4-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.2200	uM
3-[[5-fluoro-4-(3-oxo-4-phenylpiperazin-1-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.2360	uM
3-[(5-methoxy-4-piperazin-1-ylpyrimidin-2-yl)amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.2500	uM
5-cyano-N-[2-(cyclohexen-1-yl)-4-[1-[2-(dimethylamino)acetyl]piperidin-4-yl]phenyl]-1H-imidazole-2-carboxamide	1948593: Inhibition of BMPR2 (unknown origin) assessed as dissociation constant	kd	0.3100	uM
3-[[5-methoxy-4-(5-phenyl-2,5-diazabicyclo[2.2.2]octan-2-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.3100	uM
5-cyano-N-[2-(cyclohexen-1-yl)-4-[1-[2-(dimethylamino)acetyl]piperidin-4-yl]phenyl]-1H-imidazole-2-carboxamide;hydrochloride	624826: Binding constant for BMPR2 kinase domain	kd	0.3100	uM
[4-[(E)-2-(1H-indazol-3-yl)ethenyl]phenyl]-piperazin-1-ylmethanone	624826: Binding constant for BMPR2 kinase domain	kd	0.3700	uM
3-[[5-methoxy-4-[4-phenyl-2-(trifluoromethyl)piperazin-1-yl]pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.4300	uM
2,4,5,8,16,18,21-heptazatetracyclo[15.3.1.13,6.111,15]tricosa-1(21),3,6(23),11(22),12,14,17,19-octaen-7-one	1948605: Inhibition of BMPR2 (174 to end residues) (unknown origin) using MBP as substrate incubated for 60 mins in presence of ATP by ADP Glo assay	ic50	0.4610	uM
N-[3-[[5-bromo-4-[(4-sulfamoylphenyl)methylamino]pyrimidin-2-yl]amino]phenyl]pyrrolidine-1-carboxamide	1823794: Inhibition of 1recombinant human BMPR2 (172 to 734 residues) using myelin basic protein as substrate incubated for 120 mins in presence of [gamma-33P-ATP] by radiometric scintillation assay	ic50	0.4880	uM
Sunitinib	1948593: Inhibition of BMPR2 (unknown origin) assessed as dissociation constant	kd	0.5700	uM
Bosutinib	624826: Binding constant for BMPR2 kinase domain	kd	0.5800	uM
2,4,5,8,14,16,19-heptazatricyclo[13.3.1.13,6]icosa-1(19),3,6(20),15,17-pentaen-7-one	1948605: Inhibition of BMPR2 (174 to end residues) (unknown origin) using MBP as substrate incubated for 60 mins in presence of ATP by ADP Glo assay	ic50	0.6300	uM
5-[6-(4-piperazin-1-ylphenyl)pyrazolo[1,5-a]pyrimidin-3-yl]quinoline	750126: Inhibition of BMPR2 (unknown origin)	ic50	0.6950	uM
4-[[5-amino-1-(2,6-difluorobenzoyl)-1,2,4-triazol-3-yl]amino]benzenesulfonamide	435780: Binding constant for BMPR2 kinase domain	kd	0.8800	uM
3-[[5-methoxy-4-(5-phenyl-2,5-diazabicyclo[2.2.1]heptan-2-yl)pyrimidin-2-yl]amino]benzenesulfonamide	1967369: Inhibition of recombinant C-terminal 6His-tagged human BMPR2 (188 to 517 residues) expressed in Escherichia coli Rosetta assessed as apparent inhibition constant using ATP as substrate incubated for 30 mins by Morrison plot analysis	ki	0.9300	uM
Ruxolitinib	624826: Binding constant for BMPR2 kinase domain	kd	0.9300	uM
5-(3-chloroanilino)benzo[c][2,6]naphthyridine-8-carboxylic acid	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	1.0560	uM
(2S,3R,4R,6R)-3-methoxy-2-methyl-4-(methylamino)-29-oxa-1,7,17-triazaoctacyclo[12.12.2.12,6.07,28.08,13.015,19.020,27.021,26]nonacosa-8,10,12,14,19,21,23,25,27-nonaen-16-one	2198144: Inhibition of human BMPR2 using myelin basic protein as substrate preincubated for 20 mins followed by [gamma-33P]-ATP addition and measured after 120 mins by radiometric Hot-SpotSM Kinase assay	ic50	1.0700	uM
(15S,16S,18R)-16-hydroxy-16-(hydroxymethyl)-15-methyl-28-oxa-4,14,19-triazaoctacyclo[12.11.2.115,18.02,6.07,27.08,13.019,26.020,25]octacosa-1,6,8,10,12,20,22,24,26-nonaen-3-one	1948631: Binding affinity to recombinant BMPR2 (unknown origin) assessed as dissociation constant by DSF assay	kd	1.5000	uM
4-[4-amino-5-(7-methoxy-1H-indol-2-yl)imidazo[5,1-f][1,2,4]triazin-7-yl]cyclohexane-1-carboxylic acid	1424923: Kinobeads (epsilon), multiple immobilized ATP-competitive broad spectrum kinase inhibitors, used to assess residual binding of ~300 proteins simultaneously from cell lysate in the presence of a compound. Quantitative readout performed by mass spectrometry.	kd	2.0970	uM

CTD chemical–gene interactions

61 total (human), top 30 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
Benzo(a)pyrene	affects methylation, decreases expression	5
Vorinostat	decreases expression, increases expression	2
Air Pollutants	decreases expression, increases abundance	2
Arsenic	affects cotreatment, decreases expression, increases abundance, affects expression	2
Tetrachlorodibenzodioxin	affects cotreatment, increases expression	2
Tretinoin	increases expression	2
Valproic Acid	increases expression	2
Cyclosporine	decreases expression	2
aristolochic acid I	decreases expression	1
bisphenol F	increases expression	1
methylmercuric chloride	decreases expression	1
triphenyl phosphate	affects expression	1
2-butenal	decreases expression	1
arsenite	affects binding, decreases reaction	1
sodium arsenite	affects cotreatment, decreases expression, increases abundance	1
cobaltous chloride	decreases expression	1
butyraldehyde	decreases expression	1
manganese chloride	affects cotreatment, decreases expression, increases abundance	1
benzo(e)pyrene	increases methylation	1
potassium chromate(VI)	decreases expression	1
rutecarpine	decreases expression	1
2,3-bis(3’-hydroxybenzyl)butyrolactone	affects cotreatment, decreases expression	1
aflatoxin B2	affects methylation	1
5,5’-bis(8-(phenylamino)-1-naphthalenesulfonate)	affects binding, increases reaction	1
beta-methylcholine	affects expression	1
9-chloro-2-(2-furyl)-(1,2,4)triazolo(1,5-c)quinazolin-5-imine	increases expression	1
perfluorooctane sulfonic acid	decreases expression	1
kenpaullone	increases expression	1
monomethylarsonous acid	decreases expression	1
SU 9516	increases expression	1

ChEMBL screening assays

166 unique, capped per target: 165 binding, 1 admet

Representative assays (with source publication via chembl_document):

Assay ID	Type	Description	Source paper
CHEMBL1032262	Binding	Inhibition of BMPR2 at 3 uM	Discovery of substituted 4-(pyrazol-4-yl)-phenylbenzodioxane-2-carboxamides as potent and highly selective Rho kinase (ROCK-II) inhibitors. — J Med Chem
CHEMBL4377403	ADMET	Inhibition of BMPR2 (unknown origin)	Highly Selective Inhibition of Tyrosine Kinase 2 (TYK2) for the Treatment of Autoimmune Diseases: Discovery of the Allosteric Inhibitor BMS-986165. — J Med Chem

Cellosaurus cell lines

28 cell lines: 14 induced pluripotent stem cell, 8 transformed cell line, 6 cancer cell line

First 10 cell lines (id-ordered, not curated):

Cellosaurus	Name	Category	Sex
CVCL_A0YP	MHHi022-A	Induced pluripotent stem cell	Male
CVCL_A0YQ	MHHi023-A	Induced pluripotent stem cell	Female
CVCL_A0YR	MHHi024-A	Induced pluripotent stem cell	Female
CVCL_A4MB	FPAH-1-UMC	Induced pluripotent stem cell	Male
CVCL_A4MC	FPAH-1-P1	Induced pluripotent stem cell	Male
CVCL_A4MD	FPAH-1-P2	Induced pluripotent stem cell	Male
CVCL_A4ME	FPAH-1-P3	Induced pluripotent stem cell	Female
CVCL_A4MF	FPAH-2 UMC	Induced pluripotent stem cell	Female
CVCL_A4MG	FPAH-2-P	Induced pluripotent stem cell	Female
CVCL_A4MH	FPAH-3 UMC	Induced pluripotent stem cell	Male

Clinical trials (associated diseases)

380 trials via MONDO — disease-level, not drug-specific.

Trial	Phase	Status	Title
NCT00417066	PHASE4	COMPLETED	Flexible GnRH Antagonist vs Flare up GnRH Agonist Protocol in Poor Responders
NCT00732693	PHASE4	COMPLETED	Evaluation of Physiologic and Standard Sex Steroid Replacement Regimens in Women With Premature Ovarian Failure
NCT00837616	PHASE4	COMPLETED	Estrogen Dosing in Turner Syndrome: Pharmacology and Metabolism
NCT01853501	PHASE4	UNKNOWN	Effects of ADSC Therapy in Women With POF
NCT02783937	PHASE4	COMPLETED	Filgrastim for Premature Ovarian Insufficiency
NCT03535480	PHASE4	UNKNOWN	Autologous Bone Marrow Stem Cell Ovarian Transplantation to Restore Ovarian Function in Premature Ovarian Failure
NCT00058929	PHASE4	COMPLETED	A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension
NCT00303459	PHASE4	COMPLETED	Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH)
NCT00323297	PHASE4	COMPLETED	Assess the Efficacy and Safety of Sildenafil When Added to Bosentan in the Treatment of Pulmonary Arterial Hypertension
NCT00367770	PHASE4	COMPLETED	BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology
NCT00403650	PHASE4	COMPLETED	Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension
NCT00430716	PHASE4	TERMINATED	To Assess The Efficacy and Safety Of Oral Sildenafil in the Treatment of Pulmonary Arterial Hypertension.
NCT00433329	PHASE4	COMPLETED	Combination Therapy in Pulmonary Arterial Hypertension
NCT00439946	PHASE4	TERMINATED	Safety, Efficacy, and Treatment Satisfaction Switching From Flolan to Remodulin Using the Crono Five Ambulatory Pump in Patients With PAH
NCT00483626	PHASE4	UNKNOWN	Hemodynamic Response After Six Months of Sildenafil
NCT00494533	PHASE4	TERMINATED	Study of Intravenous Remodulin in Patients in India With Pulmonary Arterial Hypertension
NCT00617305	PHASE4	COMPLETED	Study of Add-on Ambrisentan Therapy to Background Phosphodiesterase Type-5 Inhibitor (PDE5i) Therapy in Pulmonary Arterial Hypertension (ATHENA-1)
NCT00625079	PHASE4	WITHDRAWN	Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil
NCT00625469	PHASE4	WITHDRAWN	Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan
NCT00705588	PHASE4	UNKNOWN	Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids.
NCT00741819	PHASE4	COMPLETED	Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects
NCT01042158	PHASE4	COMPLETED	A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis
NCT01105091	PHASE4	COMPLETED	Epoprostenol for Injection in Pulmonary Arterial Hypertension
NCT01105117	PHASE4	COMPLETED	Epoprostenol for Injection in Pulmonary Arterial Hypertension - Extension of AC-066A401
NCT01268553	PHASE4	COMPLETED	Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication
NCT01302444	PHASE4	TERMINATED	Treprostinil Combined With Tadalafil for Pulmonary Hypertension
NCT01330108	PHASE4	COMPLETED	Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension
NCT01433328	PHASE4	TERMINATED	Lidocaine Subcutaneous Infusion for Control of Treprostinil Related Site Pain
NCT01508780	PHASE4	WITHDRAWN	Combined Use of Angiography, Optical Coherence Tomography and Intravascular Ultrasound in Evaluation of Pulmonary Vascular Structure and Function in Patients With Pulmonary Arterial Hypertension Treated With Oral Bosentan
NCT01615627	PHASE4	WITHDRAWN	Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain
NCT01642407	PHASE4	COMPLETED	Safety And Efficacy Of Sildenafil In Children With Pulmonary Arterial Hypertension
NCT01649739	PHASE4	UNKNOWN	Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost
NCT02060487	PHASE4	TERMINATED	Effects of Oral Sildenafil on Mortality in Adults With PAH
NCT02253394	PHASE4	TERMINATED	The Combination Ambrisentan Plus Spironolactone in Pulmonary Arterial Hypertension Study
NCT02284737	PHASE4	TERMINATED	A Study to Investigate the Efficacy of PADN to Improved Functional Capacity and Hemodynamics in Patients With PAH
NCT02310672	PHASE4	COMPLETED	REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension
NCT02847260	PHASE4	COMPLETED	Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID)
NCT02882126	PHASE4	WITHDRAWN	An Open Label Extension Study to Evaluate the Safety of Continued Therapy of Subcutanous Remodulin® in Pulmonary Arterial Hypertension
NCT02885012	PHASE4	TERMINATED	Crossover Study From Macitentan or Bosentan Over to Ambrisentan
NCT02891850	PHASE4	COMPLETED	Riociguat rEplacing PDE-5i Therapy evaLuated Against Continued PDE-5i thErapy