PDGFD

Summary

PDGFD (platelet derived growth factor D, HGNC:30620) is a protein-coding gene on chromosome 11q22.3, encoding Platelet-derived growth factor D (Q9GZP0). Growth factor that plays an essential role in the regulation of embryonic development, cell proliferation, cell migration, survival and chemotaxis.

The protein encoded by this gene is a member of the platelet-derived growth factor family. The four members of this family are mitogenic factors for cells of mesenchymal origin and are characterized by a core motif of eight cysteines, seven of which are found in this factor. This gene product only forms homodimers and, therefore, does not dimerize with the other three family members. It differs from alpha and beta members of this family in having an unusual N-terminal domain, the CUB domain. Two splice variants have been identified for this gene.

Source: NCBI Gene 80310 — RefSeq curated summary.

At a glance

• Gene–disease (curated): pulmonary arterial hypertension (Limited, ClinGen)
• GWAS associations: 29 (top 4% of genes corpus-wide)
• Clinical variants (ClinVar): 64 total
• GTEx eGene: eQTL in 21 tissues, sQTL in 2 (q < 0.05)
• Constraint (gnomAD): pLI 0.00 (LoF-tolerant), LOEUF 0.93 (unconstrained)
• MANE Select transcript: NM_025208
• Notable: 64 clinical variants but no curated precision-oncology (CIViC) evidence yet

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:30620
Approved symbol	PDGFD
Name	platelet derived growth factor D
Location	11q22.3
Locus type	gene with protein product
Status	Approved
Aliases	SCDGF-B, MSTP036, IEGF
Ensembl gene	ENSG00000170962
Ensembl biotype	protein_coding
OMIM	609673
Entrez	80310

Gene structure

Transcript identifiers

Ensembl transcripts: 9 — 6 protein_coding, 3 nonsense_mediated_decay

MANE Select: ENST00000393158

Transcript support level (Ensembl TSL; 1 = fully mRNA-supported): TSL 1: 2, TSL 5: 1

ENST00000393158, ENST00000302251, ENST00000529268, ENST00000956141, ENST00000978581, ENST00000978582, ENST00001032629, ENST00001113424, ENST00001136224

RefSeq mRNA: 2 — MANE Select: NM_025208 NM_025208, NM_033135

CCDS: CCDS41703, CCDS8326

Canonical transcript exons

ENST00000393158 — 7 exons

Exon	Start	End
ENSE00001138232	103926912	103927126
ENSE00001138241	103943452	103943650
ENSE00001138251	103947662	103947724
ENSE00001138261	103996065	103996245
ENSE00001514313	104000051	104000255
ENSE00002157970	103907189	103909819
ENSE00002168772	104163804	104164147

Expression profiles

Bgee: expression breadth ubiquitous, 252 present calls, max score 98.41.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 6.1165 / max 111.2736, expressed in 1009 samples.

Highest expression (Bgee): periodontal ligament, germinal epithelium of ovary, seminal vesicle, adrenal cortex, ventricular zone.

Genetic regulation across tissues (GTEx eQTL / sQTL)

PDGFD is an eGene in GTEx v10 — its expression is genetically regulated (eQTL) in 21 tissues and its splicing (sQTL) in 2 (q < 0.05). Strongest associations first; slope = normalized effect of the lead variant’s alternative allele.

showing 20 of 23 significant tissue × QTL associations:

Tissue	QTL	q-value	Slope	Lead variant
Lung	eqtl	4.8e-15	+0.44	11:103841404:G:A
Pituitary	eqtl	2.1e-08	-0.50	11:103854100:C:T
Artery Aorta	eqtl	7.3e-08	-0.26	11:103802549:A:C
Cells Cultured fibroblasts	eqtl	9.0e-08	+0.10	11:104106219:G:A
Adrenal Gland	sqtl	1.6e-07	-0.83	11:103938820:C:A
Nerve Tibial	eqtl	1.3e-04	+0.52	11:104164289:G:C
Artery Aorta	sqtl	7.4e-04	-0.47	11:104000311:C:A
Pancreas	eqtl	9.9e-04	-0.42	11:103777657:G:A
Adipose Subcutaneous	eqtl	2.9e-03	-0.25	11:103992402:C:T
Heart Left Ventricle	eqtl	3.6e-03	+0.21	11:104038438:C:A
Colon Transverse	eqtl	4.7e-03	+0.19	11:104499033:G:T
Thyroid	eqtl	4.7e-03	-0.22	11:104456902:A:G
Testis	eqtl	6.7e-03	-0.31	11:104022741:T:C
Esophagus Mucosa	eqtl	8.5e-03	-0.17	11:103994207:G:A
Brain Anterior cingulate cortex BA24	eqtl	9.7e-03	-0.42	11:103337655:A:G
Artery Tibial	eqtl	1.1e-02	-0.13	11:103826123:C:T
Esophagus Muscularis	eqtl	1.4e-02	-0.17	11:104036408:T:C
Muscle Skeletal	eqtl	1.7e-02	-0.44	11:104192041:G:A
Adipose Visceral Omentum	eqtl	1.7e-02	-0.17	11:104000580:T:TG
Artery Coronary	eqtl	2.3e-02	-0.20	11:103826123:C:T

FANTOM5 promoters

7 alternative TSS:

Promoter ID	TPM avg	Samples expressed
122059	1.8042	586
122058	1.3313	479
122060	1.1517	467
122061	0.8688	409
122062	0.3988	216
122063	0.3964	227
122057	0.1653	57

Top tissues by expression

showing 100 of 282 tissues by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
periodontal ligament	UBERON:0008266	98.41	gold quality
germinal epithelium of ovary	UBERON:0001304	96.90	gold quality
seminal vesicle	UBERON:0000998	96.30	gold quality
adrenal cortex	UBERON:0001235	96.12	gold quality
ventricular zone	UBERON:0003053	96.09	gold quality
left adrenal gland cortex	UBERON:0035825	96.09	gold quality
synovial joint	UBERON:0002217	96.08	gold quality
left adrenal gland	UBERON:0001234	95.88	gold quality
right adrenal gland cortex	UBERON:0035827	95.69	gold quality
descending thoracic aorta	UBERON:0002345	95.63	gold quality
thoracic aorta	UBERON:0001515	95.43	gold quality
right adrenal gland	UBERON:0001233	95.42	gold quality
ascending aorta	UBERON:0001496	95.33	gold quality
adrenal gland	UBERON:0002369	95.04	gold quality
skin of hip	UBERON:0001554	94.10	gold quality
gall bladder	UBERON:0002110	94.02	gold quality
right coronary artery	UBERON:0001625	93.25	gold quality
mammary duct	UBERON:0001765	93.18	gold quality
stromal cell of endometrium	CL:0002255	93.14	gold quality
heart right ventricle	UBERON:0002080	92.94	gold quality
left ovary	UBERON:0002119	92.78	gold quality
tibia	UBERON:0000979	92.63	gold quality
layer of synovial tissue	UBERON:0007616	92.62	gold quality
right ovary	UBERON:0002118	92.24	gold quality
epithelium of mammary gland	UBERON:0003244	92.08	gold quality
parietal pleura	UBERON:0002400	91.89	gold quality
thoracic mammary gland	UBERON:0005200	91.50	gold quality
ovary	UBERON:0000992	91.45	gold quality
mammary gland	UBERON:0001911	91.23	gold quality
left coronary artery	UBERON:0001626	90.96	gold quality
decidua	UBERON:0002450	90.63	gold quality
coronary artery	UBERON:0001621	90.49	gold quality
adrenal tissue	UBERON:0018303	90.06	gold quality
aorta	UBERON:0000947	89.43	gold quality
upper leg skin	UBERON:0004262	88.71	gold quality
pleura	UBERON:0000977	88.65	gold quality
choroid plexus epithelium	UBERON:0003911	88.44	gold quality
body of pancreas	UBERON:0001150	86.84	gold quality
dorsal root ganglion	UBERON:0000044	86.66	gold quality
ganglionic eminence	UBERON:0004023	86.39	gold quality
urethra	UBERON:0000057	85.96	gold quality
artery	UBERON:0001637	85.56	gold quality
subcutaneous adipose tissue	UBERON:0002190	85.56	gold quality
pancreas	UBERON:0001264	85.43	gold quality
adipose tissue	UBERON:0001013	85.26	gold quality
right atrium auricular region	UBERON:0006631	85.14	gold quality
connective tissue	UBERON:0002384	84.92	gold quality
tibial artery	UBERON:0007610	84.92	gold quality
left lobe of thyroid gland	UBERON:0001120	84.91	gold quality
popliteal artery	UBERON:0002250	84.91	gold quality
thyroid gland	UBERON:0002046	84.69	gold quality
heart	UBERON:0000948	84.66	gold quality
mucosa of paranasal sinus	UBERON:0005030	84.64	gold quality
cardiac atrium	UBERON:0002081	84.63	gold quality
cardia of stomach	UBERON:0001162	84.32	gold quality
pigmented layer of retina	UBERON:0001782	84.30	gold quality
right lobe of thyroid gland	UBERON:0001119	84.19	gold quality
calcaneal tendon	UBERON:0003701	84.08	gold quality
vagina	UBERON:0000996	84.00	gold quality
parotid gland	UBERON:0001831	83.87	gold quality
omental fat pad	UBERON:0010414	83.52	gold quality
peritoneum	UBERON:0002358	83.51	gold quality
ectocervix	UBERON:0012249	83.42	gold quality
adipose tissue of abdominal region	UBERON:0007808	83.40	gold quality
pituitary gland	UBERON:0000007	83.34	gold quality
urinary bladder	UBERON:0001255	82.96	gold quality
islet of Langerhans	UBERON:0000006	82.83	gold quality
cardiac ventricle	UBERON:0002082	82.69	gold quality
uterine cervix	UBERON:0000002	82.46	gold quality
heart left ventricle	UBERON:0002084	82.44	gold quality
colonic epithelium	UBERON:0000397	82.41	gold quality
myocardium	UBERON:0002349	82.41	gold quality
visceral pleura	UBERON:0002401	82.16	gold quality
rectum	UBERON:0001052	82.11	gold quality
pylorus	UBERON:0001166	81.88	gold quality
adenohypophysis	UBERON:0002196	81.78	gold quality
endothelial cell	CL:0000115	81.50	silver quality
cauda epididymis	UBERON:0004360	81.45	gold quality
tendon	UBERON:0000043	81.32	gold quality
fundus of stomach	UBERON:0001160	81.25	gold quality
mucosa of sigmoid colon	UBERON:0004993	81.01	gold quality
apex of heart	UBERON:0002098	80.96	gold quality
tibial nerve	UBERON:0001323	80.94	gold quality
pericardium	UBERON:0002407	80.90	gold quality
cervix epithelium	UBERON:0004801	80.81	gold quality
endocervix	UBERON:0000458	80.53	gold quality
mucosa of urinary bladder	UBERON:0001259	80.43	gold quality
stomach	UBERON:0000945	80.29	gold quality
tendon of biceps brachii	UBERON:0008188	80.29	gold quality
trigeminal ganglion	UBERON:0001675	80.14	gold quality
renal glomerulus	UBERON:0000074	80.03	gold quality
male germ line stem cell (sensu Vertebrata) in testis	CL:0000089 ∩ UBERON:0000473	79.65	gold quality
body of stomach	UBERON:0001161	79.57	gold quality
granulocyte	CL:0000094	79.52	gold quality
metanephric glomerulus	UBERON:0004736	79.44	gold quality
female reproductive system	UBERON:0000474	79.39	gold quality
left uterine tube	UBERON:0001303	79.38	gold quality
saliva-secreting gland	UBERON:0001044	78.76	gold quality
minor salivary gland	UBERON:0001830	78.66	gold quality
cardiac muscle of right atrium	UBERON:0003379	78.62	gold quality

Single-cell (SCXA)

Detected in 1 experiment(s), a significant marker in 1.

Experiment	Marker?	Max mean expression
E-ANND-3	yes	8.91

HPA expression

RNA tissue specificity: Tissue enhanced

showing 60 of 76 HPA tissues / cells by nTPM:

Tissue / cell	Axis	nTPM	Protein (IHC)
Adrenal gland	tissue	100.6	not detected
Ovary	tissue	60.9	not detected
Spinal cord	tissue	51.0
Seminal vesicle	tissue	35.2	not detected
Gallbladder	tissue	31.9	not detected
Pancreas	tissue	24.2	not detected
Cervix	tissue	23.6	not detected
Stomach 1	tissue	22.1	not detected
Heart muscle	tissue	21.7	not detected
Choroid plexus	tissue	20.8
Breast	tissue	20.3	not detected
Locus coeruleus	tissue	20.2
Adipose tissue	tissue	19.0	not detected
Medulla oblongata	tissue	17.0
Thyroid gland	tissue	16.6	not detected
Parathyroid gland	tissue	16.1	not detected
Urinary bladder	tissue	14.8	not detected
Vagina	tissue	14.1	not detected
Substantia nigra	tissue	13.9
Midbrain	tissue	13.6
Pituitary gland	tissue	12.4
Olfactory bulb	tissue	11.9
Endometrium 1	tissue	11.0	not detected
Pons	tissue	10.2
Superior colliculi	tissue	9.9
Prostate	tissue	9.4	not detected
Rectum	tissue	9.3	not detected
Smooth muscle	tissue	9.1	not detected
Fallopian tube	tissue	8.7	not detected
Thalamus	tissue	8.4
Colon	tissue	8.3	not detected
Tongue	tissue	8.2
Periaqueductal gray	tissue	7.9
Salivary gland	tissue	7.6	not detected
Cerebellum	tissue	7.6	not detected
Hypothalamus	tissue	7.3
Kidney	tissue	7.2	not detected
Lymph node	tissue	6.9	not detected
Epididymis	tissue	6.7	not detected
Placenta	tissue	6.6	not detected
Tonsil	tissue	5.0	not detected
Small intestine	tissue	4.5	not detected
Corpus callosum	tissue	4.3
Frontal cortex	tissue	4.0
Putamen	tissue	4.0
Duodenum	tissue	3.9	not detected
Septum	tissue	3.6
Basal ganglia	tissue	3.5	not detected
Thymus	tissue	3.4
Caudate putamen	tissue	3.4
Ventral forebrain	tissue	3.3
Appendix	tissue	3.2	not detected
Testis	tissue	3.1	not detected
Lung	tissue	2.9	not detected
Hippocampal formation	tissue	2.9
Skin 1	tissue	2.6	not detected
Spleen	tissue	2.6	not detected
Amygdala	tissue	2.5
Cerebral cortex	tissue	2.2	not detected
Motor cortex	tissue	2.2

Brain sub-regions (HPA Brain Atlas): detected in 189; highest nTPM — Blood vessel (67.2), Ventral spinal cord (51.0), Lateral vestibular nucleus (20.8), Dorsal spinal cord (20.0), Dorsal raphe nucleus (13.5).

Regulation

Is transcription factor: no

Upstream regulators (CollecTRI, top): ETS1, IRF1, NFKB, SP1

miRNA regulators (miRDB)

Targeting PDGFD by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific).

showing 30 of 110 miRNAs:

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-5011-5P	100.00	83.46	5820
HSA-MIR-5692A	100.00	74.40	6850
HSA-MIR-6867-5P	100.00	82.21	3464
HSA-MIR-513A-5P	100.00	69.77	2465
HSA-MIR-126-5P	100.00	72.71	3180
HSA-MIR-3163	100.00	77.23	8605
HSA-MIR-9-5P	100.00	72.28	2361
HSA-MIR-3646	100.00	73.56	5283
HSA-MIR-6833-3P	100.00	70.63	3197
HSA-MIR-4795-3P	100.00	74.62	4024
HSA-MIR-656-3P	100.00	72.15	2788
HSA-MIR-4768-5P	100.00	69.49	2861
HSA-MIR-4789-5P	99.98	70.76	2721
HSA-MIR-19A-3P	99.98	75.33	2762
HSA-MIR-19B-3P	99.98	75.44	2754
HSA-MIR-4482-3P	99.98	72.50	3147
HSA-MIR-548AN	99.97	70.91	2817
HSA-MIR-3148	99.97	75.06	6478
HSA-MIR-4487	99.96	64.58	1252
HSA-MIR-3658	99.96	73.87	4379
HSA-MIR-545-3P	99.95	70.74	2783
HSA-MIR-335-3P	99.93	73.36	4958
HSA-MIR-6835-3P	99.93	70.49	2904
HSA-MIR-381-3P	99.93	71.87	2854
HSA-MIR-300	99.92	71.76	2856
HSA-MIR-450B-5P	99.92	71.48	3175
HSA-MIR-145-5P	99.92	71.13	1836
HSA-MIR-5195-3P	99.92	70.92	1877
HSA-MIR-95-5P	99.89	72.17	3973
HSA-MIR-153-5P	99.89	73.86	6317

Functional genomics

gnomAD constraint: pLI 5.65e-07, LOEUF 0.926, missense Z 0.441 — population loss-of-function intolerance (pLI ≥ 0.9 = LoF-intolerant; lower LOEUF = more constrained). gnomAD

Regional missense constraint (gnomAD RMC): no regional variation along ENST00000393158 — one region, missense o/e 0.97.

Literature-anchored findings (GeneRIF, showing 40)

• Platelet-derived growth factor D: tumorigenicity in mice and dysregulated expression in human cancer (PMID:11980634)
• we localized PDGF-D in human kidneys suggesting that PDGF-D may have a role in kidney development. (PMID:12427128)
• PDGF-D plays an important role in the pathogenesis of tubulointerstitial injury through binding of PDGF-Rbeta in both human obstructive nephropathy (PMID:14514732)
• full-length PDGF-D is activated in tissues and is capable of increasing interstitial fluid pressure and macrophage recruitment and the maturation of blood vessels in angiogenic processes. (PMID:15271796)
• PDGF D is activated and processed by urokinase plasminogen activator and they colocalize in human prostate carcinoma. (PMID:15988036)
• Sp1, like Ets-1, induces PDGF-D transcription and mRNA expression; H2O2 stimulates Ets-1, but not Sp1, and activates D3-dependent PDGF-D transcription (PMID:16189269)
• PDGF-D is present in the neointima of the arteriopathy of Chronic allograft nephropathy, where it can engage PDGF-Rbeta to promote mesenchymal cell migration, proliferation, and neointima formation. (PMID:18187181)
• All 3 residues in Sp1 (Thr668, Ser670, and Thr681) are required for Sp1-dependent platelet-derived growth factor-D activation in response to angiotensin II (PMID:18258854)
• PDGF-D promotes epithelial-mesenchymal transitiion and thus could be a novel therapeutic target for the prevention and/or treatment of prostate cancer. (PMID:18403754)
• Compared with control group, levels of PDGF-D and PDGF-B were progressively elevated in blood and urine of IgA nephropathy children. (PMID:18471357)
• PDGF-C and PDGF-D, like PDGF-A and PDGF-B, play important roles in atherosclerosis by stimulating MMP activity and influencing monocyte migration (PMID:18573494)
• Association of platelet-derived growth factor-D gene polymorphism with ischemic stroke in a Chinese case-control study (PMID:18600092)
• uPA not only generates active PDGF-DD, but also regulates its spatial distribution, provides novel insights into the biological function of PDGF-DD. (PMID:18997817)
• PDGF-DD, a novel mediator of smooth muscle cell phenotypic modulation, is upregulated in endothelial cells exposed to atherosclerosis-prone flow patterns. (PMID:19028801)
• PDGF-DD serum levels in patients with IgA nephropathy (IgAN) were significantly higher (1.67 +/- 0.45 ng/ml) and in patients with lupus nephritis significantly lower (0.66 +/- 0.86 ng/ml) compared to healthy controls. (PMID:19357108)
• PDGF-D-induced acquisition of the EMT phenotype in PC3 cells is, in part, a result of repression of miR-200. (PMID:19544444)
• LH significantly increased PDGF-D messenger RNA (mRNA) expression but suppressed PDGF-B and PDGF-C mRNA in human granulosa-luteal cells (PMID:19733850)
• human umbilical cord blood tropism toward glioma cells are partially dependent on the PDGF/PGGFR system. (PMID:20406896)
• Study unveiled a novel signaling axis of matriptase/PDGF-D/beta-PDGFR in PCa, providing new insights into functional interplay between serine protease and growth factor signaling networks. (PMID:21098708)
• The results of this study suggested that PDGFD -858A/C variant is probably associated with risk for non-hypertensive intracerebral hemorrhage. (PMID:22289441)
• PDGF-D promotes malignant mesothelioma cell chemotaxis through PDGF-betabeta receptor signaling pathways along a PI3 kinase/PDK1/Akt/Rac1/ROCK axis and relevant to ERK activation (PMID:22415093)
• High PDGFD expression promotes ovarian cancer invasion by regulating matrix metalloproteinases 2 and 9 (PMID:22471482)
• Taken together with our previous finding that matriptase is a proteolytic activator of PDGF D, this study provides a molecular insight into signal amplification of the proteolytic network and PDGF signaling loop during cancer progression. (PMID:22689130)
• Meta-analysis showed that single nucleotide polymorphism rs974819 of PDGFD gene was significantly associated with an increasing risk of coronary heart disease (OR=1.08, 95% CI=1.05 - 1.11) in both Europeans and South Asians including Han Chinese. (PMID:22704460)
• Down-regulation of platelet-derived growth factor-D expression blockades NF-kappaB pathway to inhibit cell proliferation and invasion as well as induce apoptosis in esophageal squamous cell carcinoma. (PMID:23187740)
• Platelet-derived growth factor-D and Rho GTPases regulate recruitment of cancer-associated fibroblasts in cholangiocarcinoma. (PMID:23505219)
• PDGF-D is highly expressed in gemcitabine resistant (GR) liver neoplasm cells. Furthermore, down-regulation of PDGF-D in GR cells led to partial reversal of the EMT phenotype. (PMID:24158561)
• Data suggest that targeting platelet-derived growth factor-D as a strategy for endometrial cancer treatment. (PMID:24646915)
• Compared with matched normal endometrial cases, PDGF-D was up-regulated in endometrial cancer. Expression of PDGF-D protein, found in 78% of the cases, was associated with nonendometrioid histologic type. (PMID:24870786)
• PDGF-DD secreted by gastric cancer derived mesenchymal stem cells is capable of promoting gastric cancer cell progression in vitro and in vivo. (PMID:24938433)
• PDGF-D is highly expressed by ASCs, where it acts as a potent mitogenic factor. (PMID:25332166)
• PDGFD, CDH1 and SLIT2 are upregulated in low grade meningiomas and schwannomas compared with healthy tissue. (PMID:25333347)
• PDGFD-PDGFRbeta signalling is required for human neocortical development and local production of growth factors by radial glia supports the expanded germinal region and progenitor heterogeneity of species with large brains (PMID:25391964)
• PDGF-D expression is associated with miR-106a and Twist1 in HCC patients (PMID:25760076)
• Novel findings reveal a new paradigm in matriptase activation involving PDGF-D-specific signal transduction leading to extracellular acidosis. (PMID:26157007)
• polymorphism of rs7950273 in the PDGFD gene is not associated with ischaemic stroke in the Chinese Han population (PMID:26362023)
• upregulated in kidney fibrosis, may mediate renal scarring, and is dispensable for normal kidney development and physiological functions (PMID:26924050)
• This study suggested that epithelial-mesenchymal transition process can be triggered by the PDGF-D/PDGFRb axis in tongue squamous cell carcinoma, and then involved in the tumor cell invasion via activation of p38/AKT/ERK/ epithelial-mesenchymal transition pathway. (PMID:27507215)
• No specific genotype of rs974819 of Platelet-derived growth factor D demonstrated increased cardiovascular mortality in the total population, however, the male group with genotypes A/A and G/A demonstrated an increased risk that persisted in a multivariate evaluation where adjustments were made for well-known cardiovascular risk factors. (PMID:27585990)
• PDGF-D promotes tumor growth and aggressiveness of colorectal carcinoma. Down-regulation of PDGF-D inactivates Notch1/Twist1 axis, which could reverse epithelial mesenchymal transformation and prevent CRC progression. (PMID:28035069)

Cross-species orthologs

3 orthologs

Organism	Symbol	Gene ID
danio_rerio	pdgfd	ENSDARG00000077677
mus_musculus	Pdgfd	ENSMUSG00000032006
rattus_norvegicus	Pdgfd	ENSRNOG00000029148

Paralogs (35): NRXN3 (ENSG00000021645), TLL1 (ENSG00000038295), CP (ENSG00000047457), DCBLD2 (ENSG00000057019), HEPH (ENSG00000089472), TLL2 (ENSG00000095587), NRP1 (ENSG00000099250), PCOLCE (ENSG00000106333), CNTNAP3 (ENSG00000106714), CUBN (ENSG00000107611), CNTNAP1 (ENSG00000108797), NRXN2 (ENSG00000110076), MEP1A (ENSG00000112818), NRP2 (ENSG00000118257), CUZD1 (ENSG00000138161), MFGE8 (ENSG00000140545), MEP1B (ENSG00000141434), PDGFC (ENSG00000145431), CNTNAP4 (ENSG00000152910), CNTNAP3B (ENSG00000154529), CNTNAP5 (ENSG00000155052), CDCP2 (ENSG00000157211), PCOLCE2 (ENSG00000163710), EDIL3 (ENSG00000164176), NETO1 (ENSG00000166342), BMP1 (ENSG00000168487), NETO2 (ENSG00000171208), CNTNAP2 (ENSG00000174469), NRXN1 (ENSG00000179915), HEPHL1 (ENSG00000181333), F8 (ENSG00000185010), ASTL (ENSG00000188886), F5 (ENSG00000198734), MFRP (ENSG00000235718), CNTNAP3C (ENSG00000283378)

Mouse-model phenotypes

Observed phenotypes of the mouse ortholog (MGI:1919035) from MGI (Alliance of Genome Resources) — knockout/mutant phenotypes, ranked by annotation count. 10 distinct phenotype terms.

Mouse phenotype (MP)	Records
increased circulating calcium level	1
abnormal urinary bladder morphology	1
increased body weight	1
decreased litter size	1
abnormal kidney morphology	1
decreased circulating chloride level	1
abnormal pericyte morphology	1
decreased circulating potassium level	1
increased systemic arterial diastolic blood pressure	1
increased systemic arterial systolic blood pressure	1

Additional cross-species homologs

Sequence homologs in species beyond Ensembl Compara’s model organisms, by Diamond local protein alignment (% sequence identity; best hit per species). Lower-identity rows are more distant homologs. The matched accessions’ gene symbols aren’t in biobtree yet, so each row shows the UniProt accession.

Organism	UniProt	Identity	Method
Pongo abelii	Q5RA73	99.2%	Diamond
Oryctolagus cuniculus	Q6V9H4	92.3%	Diamond
Gekko japonicus	A8WCC4	87.2%	Diamond
Gallus gallus	Q9I946	87.2%	Diamond
Sus scrofa	F1RWC3	81.9%	Diamond
Drosophila melanogaster	P25723	57.5%	Diamond

Genomic neighbors (locus context)

Genes flanking this one on the chromosome (NCBI/Entrez gene neighborhood). Positional context only — some neighbors share regulatory elements (bidirectional promoters, antisense/overlapping transcripts), but chromosomal adjacency is not itself a functional relationship (see Interactions for that).

Symbol	Type	Entrez
PDGFDDN	ncRNA	105369464
DDI1	protein-coding	414301
LINC02552	ncRNA	102723895
MIR4693	ncRNA	100616457

Protein

Protein identifiers

Platelet-derived growth factor D — Q9GZP0 (reviewed: Q9GZP0)

Alternative names: Iris-expressed growth factor, Spinal cord-derived growth factor B

All UniProt accessions (2): Q9GZP0, H0YD37

UniProt curated annotations — full annotation on UniProt →

Function. Growth factor that plays an essential role in the regulation of embryonic development, cell proliferation, cell migration, survival and chemotaxis. Potent mitogen for cells of mesenchymal origin. Plays an important role in wound healing. Induces macrophage recruitment, increased interstitial pressure, and blood vessel maturation during angiogenesis. Can initiate events that lead to a mesangial proliferative glomerulonephritis, including influx of monocytes and macrophages and production of extracellular matrix.

Subunit / interactions. Homodimer; disulfide-linked. Interacts with PDGFRB homodimers, and with heterodimers formed by PDGFRA and PDGFRB.

Subcellular location. Secreted.

Tissue specificity. Expressed at high levels in the heart, pancreas, adrenal gland and ovary and at low levels in placenta, liver, kidney, prostate, testis, small intestine, spleen and colon. In the kidney, expressed by the visceral epithelial cells of the glomeruli. A widespread expression is also seen in the medial smooth muscle cells of arteries and arterioles, as well as in smooth muscle cells of vasa rectae in the medullary area. Expressed in the adventitial connective tissue surrounding the suprarenal artery. In chronic obstructive nephropathy, a persistent expression is seen in glomerular visceral epithelial cells and vascular smooth muscle cells, as well as de novo expression by periglomerular interstitial cells and by some neointimal cells of atherosclerotic vessels. Expression in normal prostate is seen preferentially in the mesenchyme of the gland while expression is increased and more profuse in prostate carcinoma. Expressed in many ovarian, lung, renal and brain cancer-derived cell lines.

Post-translational modifications. Activated by proteolytic cleavage. Proteolytic removal of the N-terminal CUB domain releasing the core domain is necessary for unmasking the receptor-binding epitopes of the core domain. Cleavage after Arg-247 or Arg-249 by urokinase plasminogen activator gives rise to the active form.

Similarity. Belongs to the PDGF/VEGF growth factor family.

Isoforms (2)

UniProt ID	Names	Canonical?
Q9GZP0-1	1, Long, SCDGF-B-L	yes
Q9GZP0-2	2, Short, SCDGF-B-S

RefSeq proteins (2): NP_079484, NP_149126 (=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR000072	PDGF/VEGF_dom	Domain
IPR000859	CUB_dom	Domain
IPR029034	Cystine-knot_cytokine	Homologous_superfamily
IPR035914	Sperma_CUB_dom_sf	Homologous_superfamily

Pfam: PF00341, PF00431

UniProt features (16 total): disulfide bond 4, chain 2, sequence variant 2, mutagenesis site 2, site 2, signal peptide 1, splice variant 1, domain 1, glycosylation site 1

Structure

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q9GZP0-F1	73.85	0.42

Human Protein Atlas

Protein class: Predicted secreted proteins, Predicted intracellular proteins, RAS pathway related proteins

Protein evidence: Evidence at protein level

Subcellular location (HPA, imaging): golgi apparatus; vesicles

Antibody reliability (HPA): IH approved · IF approved

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 (2)

Position	Role
247–248	cleavage
249–250	cleavage

Disulfide bonds (4)

Type	Positions
Disulfide bond	306–362, 109–131, 302–360
Interchain	296

Glycosylation sites (1)

Type	Positions
N-linked (glcnac…) asparagine	276

Mutagenesis-validated functional residues (2)

Position	Phenotype
247	abolishes cleavage into active form; when associated with a-249.
249	abolishes cleavage into active form; when associated with a-247.

Function

Pathways and Gene Ontology

Reactome pathways

1 curated (TAS) of 3; 0 disease pathways. Curated-first; evidence = TAS (manually curated) vs IEA (electronic inference).

3 pathways:

ID	Pathway	Evidence
R-HSA-186797	Signaling by PDGF	curated
R-HSA-162582	Signal Transduction	—
R-HSA-9006934	Signaling by Receptor Tyrosine Kinases	—

MSigDB gene sets: 261 (showing top): VALK_AML_WITH_FLT3_ITD, GOBP_PLATELET_DERIVED_GROWTH_FACTOR_RECEPTOR_SIGNALING_PATHWAY, WAMUNYOKOLI_OVARIAN_CANCER_LMP_DN, MULLIGHAN_NPM1_SIGNATURE_3_UP, GOBP_MYELOID_LEUKOCYTE_MIGRATION, GOBP_CELL_CHEMOTAXIS, BERTUCCI_MEDULLARY_VS_DUCTAL_BREAST_CANCER_DN, GOBP_RESPONSE_TO_ACID_CHEMICAL, chr11q22, XU_HGF_TARGETS_REPRESSED_BY_AKT1_DN, GOBP_POSITIVE_REGULATION_OF_FIBROBLAST_PROLIFERATION, GOBP_POSITIVE_REGULATION_OF_CELLULAR_EXTRAVASATION, GOBP_POSITIVE_REGULATION_OF_MAPK_CASCADE, GOMF_GROWTH_FACTOR_ACTIVITY, GOBP_MUSCLE_CELL_PROLIFERATION

GO Biological Process (14): positive regulation of cell population proliferation (GO:0008284), positive regulation of cell migration (GO:0030335), cellular response to platelet-derived growth factor stimulus (GO:0036120), platelet-derived growth factor receptor signaling pathway (GO:0048008), positive regulation of fibroblast proliferation (GO:0048146), positive regulation of smooth muscle cell proliferation (GO:0048661), positive regulation of cell division (GO:0051781), positive regulation of phosphatidylinositol 3-kinase/protein kinase B signal transduction (GO:0051897), cellular response to hydrogen peroxide (GO:0070301), positive regulation of ERK1 and ERK2 cascade (GO:0070374), cellular response to transforming growth factor beta stimulus (GO:0071560), positive regulation of smooth muscle cell chemotaxis (GO:0071673), positive regulation of glomerular mesangial cell proliferation (GO:0072126), positive regulation of monocyte extravasation (GO:2000439)

GO Molecular Function (2): platelet-derived growth factor receptor binding (GO:0005161), growth factor activity (GO:0008083)

GO Cellular Component (4): Golgi membrane (GO:0000139), extracellular region (GO:0005576), endoplasmic reticulum lumen (GO:0005788), membrane (GO:0016020)

Reactome top-level categories

2 top-level categories:

Category	Pathways
Signaling by Receptor Tyrosine Kinases	1
Signal Transduction	1

GO top-level categories

43 GO namespaces:

Category	Terms
positive regulation of cellular process	2
cellular response to growth factor stimulus	2
positive regulation of cell population proliferation	2
cellular anatomical structure	2
cell population proliferation	1
regulation of cell population proliferation	1
cell migration	1
regulation of cell migration	1
positive regulation of cell motility	1
response to platelet-derived growth factor	1
cell surface receptor protein tyrosine kinase signaling pathway	1
fibroblast proliferation	1
regulation of fibroblast proliferation	1
smooth muscle cell proliferation	1
regulation of smooth muscle cell proliferation	1
cell division	1
regulation of cell division	1
phosphatidylinositol 3-kinase/protein kinase B signal transduction	1
regulation of phosphatidylinositol 3-kinase/protein kinase B signal transduction	1
positive regulation of intracellular signal transduction	1
cellular response to reactive oxygen species	1
response to hydrogen peroxide	1
positive regulation of MAPK cascade	1
ERK1 and ERK2 cascade	1
regulation of ERK1 and ERK2 cascade	1
response to transforming growth factor beta	1
positive regulation of smooth muscle cell migration	1
positive regulation of chemotaxis	1
smooth muscle cell chemotaxis	1
regulation of smooth muscle cell chemotaxis	1
glomerular mesangial cell proliferation	1
regulation of glomerular mesangial cell proliferation	1
positive regulation of cell proliferation involved in kidney development	1
positive regulation of cellular extravasation	1
monocyte extravasation	1
positive regulation of mononuclear cell migration	1
regulation of monocyte extravasation	1
growth factor receptor binding	1
receptor ligand activity	1
Golgi apparatus	1
bounding membrane of organelle	1
endoplasmic reticulum	1
intracellular organelle lumen	1

Protein interactions and networks

STRING

showing 15 of 1,110 interactions by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
PDGFD	PDGFRB	P09619	829
PDGFD	PDGFRA	P16234	813
PDGFD	PDGFB	P01127	807
PDGFD	PDGFA	P04085	763
PDGFD	FLT1	P16057	680
PDGFD	VEGFC	P49767	597
PDGFD	PDGFC	Q9NRA1	582
PDGFD	VEGFD	O43915	547
PDGFD	NOTCH3	Q9UM47	444
PDGFD	DLL1	O00548	436
PDGFD	TGFB1	P01137	423
PDGFD	PDGFRL	Q15198	394
PDGFD	EGF	P01133	385
PDGFD	ST14	Q9Y5Y6	385
PDGFD	DNAAF10	Q96MX6	384

IntAct

Human interaction partners of PDGFD in IntAct, one row per partner (best MIscore; records = supporting IntAct interaction records).

1 partners by confidence:

Partner	Interaction type	MIscore	Records
DCTN6	association	0.530	3

BioGRID (38 interactions; top human partners by supporting records): ACTR10 (Affinity Capture-MS; 2 records), ACTR1B (Affinity Capture-MS; 2 records), DCTN1 (Affinity Capture-MS; 2 records), DCTN3 (Affinity Capture-MS; 2 records), DCTN4 (Affinity Capture-MS; 2 records), DCTN5 (Affinity Capture-MS; 2 records), DCTN6 (Affinity Capture-MS; 2 records), ECI2 (Affinity Capture-MS; 2 records), LRP2 (Affinity Capture-MS; 2 records), MAEA (Affinity Capture-MS; 2 records), NEURL4 (Affinity Capture-MS; 2 records), POTEE (Affinity Capture-MS; 2 records), SEH1L (Affinity Capture-MS; 2 records), UBR1 (Affinity Capture-MS; 2 records), UBR5 (Affinity Capture-MS; 2 records)

Ligand–receptor pairs (CellPhoneDB) (2)

Curated ligand–receptor partners (cell-cell communication), distinct from the protein–protein interactions above.

2 ligand–receptor pairs:

Partner	This gene’s role	Signaling class
PDGFRA;PDGFRB	ligand	Signaling by Platelet-derived growth factor
PDGFRB	ligand	Signaling by Platelet-derived growth factor

Disease & clinical

Genetic variants

Variant evidence for PDGFD: 64 ClinVar records (0 pathogenic / likely pathogenic); 29 GWAS associations.

ClinVar

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

Classification	Count (floor)
Pathogenic	0
Likely pathogenic	0
Uncertain significance	53
Likely benign	2
Benign	0

SpliceAI

Top 10 splice-impact sites by Δscore (adjacent positions with the same effect merged into one range; 32 predictions of 1,491):

Position(s)	Effect	Δscore	Sites
11:103943450–103943451	donor_loss	1.0000	4
11:103947725–103947726	acceptor_loss	1.0000	6
11:103996246	acceptor_loss	1.0000
11:103996254–103996256	acceptor_gain	1.0000	2
11:104000299	acceptor_gain	1.0000
11:104000303	acceptor_gain	1.0000
11:103927127–103927128	acceptor_loss	0.9900	6
11:103943603	acceptor_gain	0.9900
11:103947660–103947661	donor_loss	0.9900	5
11:103996063–103996064	donor_loss	0.9900	5

AlphaMissense

834 missense substitutions predicted likely pathogenic (of 2,428 scored), across 199 residues. Top 20 hotspot residues, ranked by the number of likely-pathogenic substitutions (of those reachable by a single-nucleotide change), then by mean score:

Residue	Likely-pathogenic substitutions	Mean am_pathogenicity	Max am_pathogenicity
D112	8	0.997	0.999
D154	8	0.981	0.996
F151	8	0.979	0.999
F163	8	0.977	0.999
F95	8	0.972	0.996
F99	8	0.959	0.996
H356	8	0.939	0.970
S66	8	0.932	0.999
F293	8	0.918	0.991
H328	8	0.914	0.991
D118	8	0.913	0.988
F157	8	0.903	0.984
H357	8	0.886	0.930
D351	8	0.865	0.953
N276	8	0.860	0.977
C109	7	0.998	0.999
C131	7	0.996	0.998
C272	7	0.996	0.998
C296	7	0.996	0.999
C302	7	0.995	0.998

HPA cancer prognostics

RNA cancer specificity: Cancer enhanced (Kidney Renal Clear Cell Carcinoma)

Human Protein Atlas survival analysis — favorable = higher expression predicts better survival.

4 prognostic cancer types:

Cancer	Prognostic	p-value
Kidney Renal Clear Cell Carcinoma (TCGA)	favorable	4.9e-12
Kidney Renal Clear Cell Carcinoma (validation)	favorable	4.47e-05
Ovary Serous Cystadenocarcinoma (validation)	unfavorable	0.000242
Glioblastoma Multiforme (validation)	unfavorable	0.000263

Disease associations

OMIM: gene MIM:609673 | disease phenotypes:

GenCC curated gene-disease

1 curated gene-disease records:

Disease	Classification	Inheritance	Records
pulmonary arterial hypertension	Limited	Autosomal dominant

ClinGen Gene-Disease Validity

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

1 validity classifications:

Disease	Classification	Inheritance
pulmonary arterial hypertension	Limited	AD

Mondo (1): pulmonary arterial hypertension (MONDO:0015924)

GWAS associations

29 associations:

Study	Trait	p-value
GCST006585_2900	Blood protein levels	3e-129
GCST011365_99	Myocardial infarction	2e-18
GCST004787_72	Coronary artery disease (myocardial infarction, percutaneous transluminal coronary angioplasty, coronary artery bypass grafting, angina or chromic ischemic heart disease)	2e-11
GCST011364_18	Myocardial infarction	3e-11
GCST010204_137	Low density lipoprotein cholesterol levels	4e-11
GCST003116_3	Coronary artery disease	7e-11
GCST011616_56	Cortical volume	8e-11
GCST010225_25	Cortical surface area (visual PC2)	3e-10
GCST005924_1	Corticosteroid-induced adrenal suppression (peak cortisol < 500 nmol/L)	4e-10
GCST010245_28	LDL cholesterol levels	1e-9
GCST000999_11	Coronary heart disease	2e-9
GCST010480_9	Coronary artery disease	3e-9
GCST003117_16	Myocardial infarction	4e-9
GCST005927_1	Corticosteroid-induced adrenal suppression (peak cortisol < 350 nmol/L)	4e-9
GCST010241_195	Apolipoprotein A1 levels	4e-9
GCST010242_438	HDL cholesterol levels	1e-8
GCST006810_20	Self-reported risk-taking behaviour	2e-8
GCST010479_48	Coronary artery disease	2e-8
GCST010243_222	Apolipoprotein B levels	4e-8
GCST006248_2	Response to lurasidone in schizophrenia	2e-7
GCST90017148_1	Brain asymmetrical skew (vertical)	2e-7
GCST010397_80	Gut microbiota (bacterial taxa, rank normal transformation method)	4e-7
GCST010736_8	Urinary albumin-to-creatinine ratio	4e-7
GCST001059_15	Neutrophil count	6e-7
GCST002671_10	Toenail selenium levels	2e-6
GCST002875_121	Diisocyanate-induced asthma	2e-6
GCST000975_3	LDL cholesterol	3e-6
GCST009391_1845	Metabolite levels	8e-6
GCST90000015_9	Parkinson’s disease motor subtype (tremor to postural instability/gait difficulty score ratio)	8e-6

EFO canonical traits (from GWAS)

14 canonical traits:

EFO ID	Trait name
EFO:0004611	low density lipoprotein cholesterol measurement
EFO:0004833	neutrophil count
EFO:0006995	response to diisocyanate
EFO:0009176	adrenal suppression measurement
EFO:0008579	risk-taking behaviour
EFO:0010341	cholesteryl ester 16:0 measurement
EFO:0004771	visual cortical surface area measurement
EFO:0004614	apolipoprotein A 1 measurement
EFO:0004612	high density lipoprotein cholesterol measurement
EFO:0004615	apolipoprotein B measurement
EFO:0007874	gut microbiome measurement
EFO:0007778	urinary albumin to creatinine ratio
EFO:0600011	Parkinson’s disease symptom measurement
EFO:0004346	neuroimaging measurement

MeSH disease descriptors

1 disease descriptors:

Descriptor	Name	Tree numbers
D000081029	Pulmonary Arterial Hypertension	C08.381.423.847

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

PharmGKB: 1 entry (CPIC guideline: no)

CTD chemical–gene interactions

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

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	affects expression, increases expression	5
sodium arsenite	decreases expression, increases expression	3
bisphenol A	affects cotreatment, increases methylation, increases expression, affects methylation	2
Air Pollutants	increases abundance, decreases expression, affects expression	2
Doxorubicin	decreases expression, increases expression	2
8-Bromo Cyclic Adenosine Monophosphate	decreases expression, increases expression	2
Aflatoxin B1	decreases expression, decreases methylation	2
Particulate Matter	affects expression, increases abundance, decreases expression	2
bisphenol F	affects cotreatment, affects expression	1
ammonium 2,3,3,3-tetrafluoro-2-(heptafluoropropoxy)-propanoate	affects cotreatment, affects expression	1
methylmercuric chloride	decreases expression	1
triphenyl phosphate	affects expression	1
trichostatin A	decreases expression, increases expression	1
arsenite	increases methylation	1
mono-(2-ethylhexyl)phthalate	decreases expression	1
butyraldehyde	decreases expression	1
perfluorooctanoic acid	affects cotreatment, affects expression	1
di-n-butylphosphoric acid	affects expression	1
perfluorooctane sulfonic acid	affects expression, affects cotreatment	1
CGP 52608	affects binding, increases reaction	1
perfluoro-n-nonanoic acid	decreases expression	1
4-(5-benzo(1,3)dioxol-5-yl-4-pyridin-2-yl-1H-imidazol-2-yl)benzamide	decreases expression, decreases reaction	1
4(2’-aminoethyl)amino-1,8-dimethylimidazo(1,2-a)quinoxaline	decreases expression, decreases reaction	1
abrine	decreases expression	1
perfluorobutanesulfonic acid	affects expression, affects cotreatment	1
asparanin A	decreases expression	1
incobotulinumtoxinA	increases expression	1
Temozolomide	increases expression	1
Fulvestrant	affects cotreatment, increases methylation	1
Arsenic	affects expression	1

Cellosaurus cell lines

2 cell lines: 2 cancer cell line

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

Cellosaurus	Name	Category	Sex
CVCL_B2AA	Abcam HeLa PDGFD KO	Cancer cell line	Female
CVCL_D7WP	Ubigene A-549 PDGFD KO	Cancer cell line	Male

Clinical trials (associated diseases)

Via MONDO — disease-level, not drug-specific.

showing 60 of 300 trials:

NCT	Phase	Status	Title
NCT00058929	Phase 4	COMPLETED	A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension
NCT00303459	Phase 4	COMPLETED	Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH)
NCT00323297	Phase 4	COMPLETED	Assess the Efficacy and Safety of Sildenafil When Added to Bosentan in the Treatment of Pulmonary Arterial Hypertension
NCT00367770	Phase 4	COMPLETED	BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology
NCT00403650	Phase 4	COMPLETED	Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension
NCT00430716	Phase 4	TERMINATED	To Assess The Efficacy and Safety Of Oral Sildenafil in the Treatment of Pulmonary Arterial Hypertension.
NCT00433329	Phase 4	COMPLETED	Combination Therapy in Pulmonary Arterial Hypertension
NCT00439946	Phase 4	TERMINATED	Safety, Efficacy, and Treatment Satisfaction Switching From Flolan to Remodulin Using the Crono Five Ambulatory Pump in Patients With PAH
NCT00483626	Phase 4	UNKNOWN	Hemodynamic Response After Six Months of Sildenafil
NCT00494533	Phase 4	TERMINATED	Study of Intravenous Remodulin in Patients in India With Pulmonary Arterial Hypertension
NCT00617305	Phase 4	COMPLETED	Study of Add-on Ambrisentan Therapy to Background Phosphodiesterase Type-5 Inhibitor (PDE5i) Therapy in Pulmonary Arterial Hypertension (ATHENA-1)
NCT00625079	Phase 4	WITHDRAWN	Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil
NCT00625469	Phase 4	WITHDRAWN	Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan
NCT00705588	Phase 4	UNKNOWN	Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids.
NCT00741819	Phase 4	COMPLETED	Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects
NCT01042158	Phase 4	COMPLETED	A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis
NCT01105091	Phase 4	COMPLETED	Epoprostenol for Injection in Pulmonary Arterial Hypertension
NCT01105117	Phase 4	COMPLETED	Epoprostenol for Injection in Pulmonary Arterial Hypertension - Extension of AC-066A401
NCT01268553	Phase 4	COMPLETED	Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication
NCT01302444	Phase 4	TERMINATED	Treprostinil Combined With Tadalafil for Pulmonary Hypertension
NCT01330108	Phase 4	COMPLETED	Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension
NCT01433328	Phase 4	TERMINATED	Lidocaine Subcutaneous Infusion for Control of Treprostinil Related Site Pain
NCT01508780	Phase 4	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	Phase 4	WITHDRAWN	Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain
NCT01642407	Phase 4	COMPLETED	Safety And Efficacy Of Sildenafil In Children With Pulmonary Arterial Hypertension
NCT01649739	Phase 4	UNKNOWN	Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost
NCT02060487	Phase 4	TERMINATED	Effects of Oral Sildenafil on Mortality in Adults With PAH
NCT02253394	Phase 4	TERMINATED	The Combination Ambrisentan Plus Spironolactone in Pulmonary Arterial Hypertension Study
NCT02284737	Phase 4	TERMINATED	A Study to Investigate the Efficacy of PADN to Improved Functional Capacity and Hemodynamics in Patients With PAH
NCT02310672	Phase 4	COMPLETED	REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension
NCT02847260	Phase 4	COMPLETED	Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID)
NCT02882126	Phase 4	WITHDRAWN	An Open Label Extension Study to Evaluate the Safety of Continued Therapy of Subcutanous Remodulin® in Pulmonary Arterial Hypertension
NCT02885012	Phase 4	TERMINATED	Crossover Study From Macitentan or Bosentan Over to Ambrisentan
NCT02891850	Phase 4	COMPLETED	Riociguat rEplacing PDE-5i Therapy evaLuated Against Continued PDE-5i thErapy
NCT02893995	Phase 4	WITHDRAWN	Safety, Tolerability, Pharmacokinetics and Efficacy of Two Different Rates of Subcutanous Remodulin® Dose Titration in Pulmonary Arterial Hypertension
NCT02968901	Phase 4	TERMINATED	Clinical Study Evaluating the Effects of First-line Oral cOmbination theraPy of maciTentan and tadalafIl in Patients With Newly Diagnosed pulMonary Arterial Hypertension (OPTIMA)
NCT03055221	Phase 4	COMPLETED	TRUST-2: Safety and Efficacy of Intravenous Remodulin® in Patients in India With Pulmonary Arterial Hypertension (PAH)
NCT03078907	Phase 4	COMPLETED	Effect of Selexipag on Daily Life Physical Activity of Patients With Pulmonary Arterial Hypertension.
NCT03236818	Phase 4	UNKNOWN	Goal Oriented Strategy to Preserve Ejection Fraction Trial
NCT03344159	Phase 4	COMPLETED	Spironolactone Therapy in Chronic Stable Right HF Trial
NCT03497689	Phase 4	COMPLETED	EXPEDITE: A Study of Remodulin Induction Followed by Orenitram Optimization to Treat Pulmonary Arterial Hypertension
NCT04435782	Phase 4	TERMINATED	A Study of Selexipag Assessing Right Ventricular Remodeling in Pulmonary Arterial Hypertension by Cardiac Magnetic Resonance Imaging
NCT04955990	Phase 4	TERMINATED	A Study of Real-world Cohort of Pulmonary Arterial Hypertension (PAH) Participants
NCT05203510	Phase 4	ACTIVE_NOT_RECRUITING	A Study of a Mean Pulmonary Artery Pressure-Targeted Approach With Early and Rapid Treprostinil Therapy to Reverse Right Ventricular Remodeling in Participants With Pulmonary Arterial Hypertension
NCT05439460	Phase 4	COMPLETED	Management of Acute Pulmonary Hypertensive Crisis in Children With Known Pulmonary Arterial Hypertension
NCT05825417	Phase 4	ACTIVE_NOT_RECRUITING	Pulmonary Hypertension: Intensification and Personalisation of Combination Rx
NCT05828147	Phase 4	UNKNOWN	Anti-CD20 Antibodies for Treatment of SLE-PAH
NCT06025916	Phase 4	RECRUITING	Postpartum Hemorrhage Reduction With Oral Tranexamic Acid: a Clinical Trial
NCT06317805	Phase 4	RECRUITING	Initial Triple Therapy Including Parenteral Treprostinil vs Initial Double Oral Therapy in PAH Group I Patients
NCT06409026	Phase 4	COMPLETED	Sotatercept on Central Cardiopulmonary Performance and Peripheral Oxygen Transport During Exercise in Pulmonary Arterial Hypertension
NCT06605326	Phase 4	COMPLETED	Subcutaneous Treprostinil as a Bridge to Lung Transplantation in Severe Pulmonary Hypertension: A Single-Arm Retrospective Study
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