EDNRA

Summary

EDNRA (endothelin receptor type A, HGNC:3179) is a protein-coding gene on chromosome 4q31.22-q31.23, encoding Endothelin-1 receptor (P25101). Receptor for endothelin-1.

This gene encodes the receptor for endothelin-1, a peptide that plays a role in potent and long-lasting vasoconstriction. This receptor associates with guanine-nucleotide-binding (G) proteins, and this coupling activates a phosphatidylinositol-calcium second messenger system. Polymorphisms in this gene have been linked to migraine headache resistance. Alternative splicing results in multiple transcript variants.

Source: NCBI Gene 1909 — RefSeq curated summary.

At a glance

• Gene–disease (curated): mandibulofacial dysostosis with alopecia (Definitive, GenCC) — +1 more curated relationship
• GWAS associations: 36
• Clinical variants (ClinVar): 143 total — 2 pathogenic
• Phenotypes (HPO): 67
• Druggable target: yes — 31 molecules with ChEMBL bioactivity
• MANE Select transcript: NM_001957

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:3179
Approved symbol	EDNRA
Name	endothelin receptor type A
Location	4q31.22-q31.23
Locus type	gene with protein product
Status	Approved
Aliases	ET-A, ETA-R, hET-AR
Ensembl gene	ENSG00000151617
Ensembl biotype	protein_coding
OMIM	131243
Entrez	1909

Gene structure

Transcript identifiers

Ensembl transcripts: 11 — 8 protein_coding, 2 protein_coding_CDS_not_defined, 1 nonsense_mediated_decay

ENST00000324300, ENST00000358556, ENST00000503721, ENST00000506066, ENST00000510697, ENST00000511804, ENST00000514245, ENST00000648866, ENST00000651419, ENST00000932258, ENST00000942102

RefSeq mRNA: 2 — MANE Select: NM_001957 NM_001166055, NM_001957

CCDS: CCDS3769, CCDS54810

Canonical transcript exons

ENST00000651419 — 8 exons

Exon	Start	End
ENSE00001000667	147485612	147486101
ENSE00003527595	147539817	147539950
ENSE00003598374	147519851	147519978
ENSE00003612744	147532506	147532704
ENSE00003644644	147535877	147536029
ENSE00003676515	147540377	147540485
ENSE00003841325	147481097	147481376
ENSE00003889050	147542478	147544954

Expression profiles

Bgee: expression breadth ubiquitous, 253 present calls, max score 98.06.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 8.0812 / max 476.0221, expressed in 1029 samples.

FANTOM5 promoters (7 alternative TSS)

Promoter ID	TPM avg	Samples expressed
49933	4.0358	904
49930	1.4645	407
49932	1.2253	534
49929	0.8945	391
49931	0.2695	127
49934	0.1740	75
49928	0.0176	6

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
cauda epididymis	UBERON:0004360	98.06	gold quality
seminal vesicle	UBERON:0000998	97.45	gold quality
visceral pleura	UBERON:0002401	95.42	gold quality
urethra	UBERON:0000057	94.61	gold quality
endometrium	UBERON:0001295	94.60	gold quality
caput epididymis	UBERON:0004358	94.23	gold quality
cardiac muscle of right atrium	UBERON:0003379	94.05	gold quality
tibia	UBERON:0000979	93.94	gold quality
corpus epididymis	UBERON:0004359	92.98	gold quality
stromal cell of endometrium	CL:0002255	92.92	gold quality
uterus	UBERON:0000995	92.37	gold quality
cartilage tissue	UBERON:0002418	92.23	gold quality
myometrium	UBERON:0001296	91.95	gold quality
endocervix	UBERON:0000458	91.66	gold quality
blood vessel layer	UBERON:0004797	91.44	gold quality
ectocervix	UBERON:0012249	90.87	gold quality
pleura	UBERON:0000977	90.62	gold quality
left uterine tube	UBERON:0001303	90.48	gold quality
saphenous vein	UBERON:0007318	90.48	gold quality
skin of hip	UBERON:0001554	90.27	gold quality
decidua	UBERON:0002450	89.91	gold quality
body of uterus	UBERON:0009853	89.85	gold quality
right ovary	UBERON:0002118	89.83	gold quality
right coronary artery	UBERON:0001625	89.71	gold quality
smooth muscle tissue	UBERON:0001135	89.55	gold quality
periodontal ligament	UBERON:0008266	89.41	gold quality
cardiac atrium	UBERON:0002081	89.38	gold quality
female reproductive system	UBERON:0000474	89.37	gold quality
myocardium	UBERON:0002349	89.35	gold quality
right atrium auricular region	UBERON:0006631	88.91	gold quality

Single-cell (SCXA)

Detected in 7 experiment(s), a significant marker in 7.

Experiment	Marker?	Max mean expression
E-ANND-5	yes	548.63
E-HCAD-10	yes	44.23
E-HCAD-11	yes	20.71
E-CURD-119	yes	12.23
E-MTAB-5061	yes	11.40
E-ANND-3	yes	9.60
E-ENAD-27	yes	6.48

Regulation

Is transcription factor: no

Upstream regulators (CollecTRI, top): AP1, ESR1, HOXA10, PARP1, PITX2

miRNA regulators (miRDB)

152 targeting EDNRA, 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-30A-5P	100.00	76.31	3233
HSA-MIR-30B-5P	100.00	76.29	3248
HSA-MIR-30C-5P	100.00	76.29	3248
HSA-MIR-30D-5P	100.00	76.32	3233
HSA-MIR-30E-5P	100.00	76.32	3242
HSA-MIR-8485	100.00	77.57	4731
HSA-MIR-5011-5P	100.00	83.46	5820
HSA-MIR-4455	100.00	65.48	1587
HSA-MIR-3163	100.00	77.23	8605
HSA-MIR-190A-3P	100.00	80.35	5520
HSA-MIR-4692	100.00	67.32	2066
HSA-MIR-3689D	100.00	66.14	1181
HSA-MIR-6851-5P	100.00	65.63	1294
HSA-MIR-200B-3P	100.00	73.31	2693
HSA-MIR-200C-3P	100.00	73.35	2685
HSA-MIR-429	100.00	73.44	2698
HSA-MIR-3613-3P	100.00	76.36	7965
HSA-MIR-5692A	100.00	74.40	6850
HSA-MIR-6748-5P	100.00	65.81	1057
HSA-MIR-511-3P	99.99	68.85	1467
HSA-MIR-6759-5P	99.99	66.54	785
HSA-MIR-4514	99.99	67.10	1870
HSA-MIR-485-3P	99.98	70.68	1585
HSA-MIR-539-3P	99.98	70.74	1616
HSA-MIR-5696	99.98	72.36	4487
HSA-MIR-27A-3P	99.98	72.13	2955
HSA-MIR-27B-3P	99.98	72.13	2955
HSA-MIR-9985	99.98	72.11	2939
HSA-MIR-548N	99.98	71.94	4170
HSA-MIR-607	99.97	73.62	5593

Literature-anchored findings (GeneRIF, showing 40)

• The endothelin system plays a role in the complex pathophysiology of idiopathic dilated cardiomyopathy. (PMID:11601839)
• The vasoconstrictor effect of endothelin-1 in small resistance arteries of normotensive subjects and, in part, also in hypertensive patients is mediated by ET(A) receptors, while ET(B) receptors play a minor role, if any (PMID:11930911)
• endothelin-1 increased diastolic distensibility of acutely loaded myocardium and this effect was mediated by endothelin (A) receptors and Na(+)/H(+) exchanger activation (PMID:12595285)
• In autosomal-dominant polycystic kidney disease, neo-expression of ETA receptors has been found overlying glomeruli and cysts and markedly increased in medium-sized renal arteries. (PMID:12629276)
• Data show that in a human Kaposi sarcoma cell line, blockade of endothelin receptors A and B blocked the endothelin-1-induced increase in secretion and activation of matrix-metalloproteinases. (PMID:12875994)
• ET(A)R expression in breast cancer corrrelated with favorable prognosis. (PMID:14519635)
• It is clear from the present data for lung fibroblasts that for both stimulated and unstimulated states of ETA receptors, there exist multiple covalent forms differing in the number and location of sites of posttranslational modifications. (PMID:14636059)
• Endothelin ETA receptor mRNA levels are significantly higher in arteries from patients with ischemic heart disease as compared to congestive heart failure and controls. (PMID:14729387)
• there is an increased microvessel density (MVD) in metastatic bone lesions from different primary tumors, and ETA and ET1 are upregulated in metastatic bone lesions (PMID:15041798)
• ET-1 induces collagen matrix contraction through the ETA, but not the ETB, receptor (PMID:15047866)
• increased ET-1, ET(A)R, and ET(B)R expression are associated with increased VEGF expression and higher vascularity of breast carcinomas and could be involved in the regulation of angiogenesis in breast cancer (PMID:15073116)
• Endothelin receptor type A was significantly down-regulated in papillary renal tumor specimens (PMID:15139053)
• endothelin A receptor regulates cell migration through the Cdc42-dependent c-Jun N-terminal kinase pathway and is mediated by Nck1 (PMID:15187089)
• ET-1/ETA/ETB autocrine/paracrine loops on DCs appear to be essential for the normal maturation and function of human dendritic cells (PMID:15213100)
• Recycling of ET(A) receptor is mediated by a motif with the structural characteristics of an internal PDZ ligand. This structural motif may represent a more general principle of endocytic sorting of G protein-coupled receptors. (PMID:15713850)
• Examine therapeutic targeting of the endothelin-A receptor in human ovarian carcinoma. Report that efficacy of cytotoxic agents is markedly enhanced by co-administration with atrasentan. (PMID:15838262)
• Compared kidney endothelin-A and endothelin-B receptor distribution visualized by radioligand binding versus immunocytochemical localization using subtype selective antisera. (PMID:15838285)
• Results suggest that [F]-SB209670 binds rapidly and primarily to endothelin-A receptors in the heart. (PMID:15838315)
• Report pharmacological characterization of YM598, a selective endothelin-A receptor antagonist. (PMID:15838329)
• Targeting the endothelin-A receptor, but not the endothelin-B receptor, may be a valid tool in the therapy of cervical carcinoma. (PMID:15838364)
• Report the effects of the selective ET(A) receptor antagonist, sitaxsentan sodium, in a patient population with pulmonary arterial hypertension that meets traditional inclusion criteria of previous pulmonary arterial hypertension trials (PMID:15838366)
• Endothelin-A receptor antagonism promotes decreased vasodilation but has no differential effect on coronary artery compliance in hypertensive patients. (PMID:15838367)
• The polymorphism of EDNRA/C+70G may be related to NTG (normal tension glaucoma) risk factors. (PMID:15988412)
• Polymorphisms in the ET-1 gene (K198N), the ET receptor type A (ETA), (-231G>A and +1222C>T), and the ET type B (ETB) receptor (G57S and L277L) do not play a role in cerebral small-vessel disease (PMID:16002759)
• in human lung fibroblasts ET-1 exerts a profibrogenic action via an ET(A) receptor-dependent, MAPK-mediated induction of IL-11 release and cell proliferation (PMID:16149067)
• Observations strongly suggest that the expression of ETA receptor is enhanced in neointimal smooth muscle cells at early stages after percutaneous coronary intervention injury in human coronary arteries. (PMID:16531800)
• Integrin-linked kinase functions as a downstream mediator of the ET-1/ET(A)R axis to potentiate aggressive cellular behavior in ovarian cancer cells. (PMID:16648553)
• Reverse transcriptase-polymerase chain reaction analysis revealed mRNA the ETA receptor subtype in the human trigeminal ganglion. Immunocytochemistry revealed numerous cell bodies containing ETA proteins. (PMID:16816835)
• endothelin receptor type A(ETR-A) is selectively up-regulated in placental tissue of delayed miscarriages as compared to normal pregnancies (PMID:16879994)
• Associations between endothelin receptors A and B in aystemic ssclerosis subsets supports the role of endothelin and its receptors in the pathogenesis of this disease. (PMID:16947775)
• ET receptors in pigment cells of vertebrate species were identified by RT-PCR assays, and the differential expression of the various subtypes in each species was compared by quantitative PCR. (PMID:16962346)
• Polymorphism of EDNRA:c.*1222C > T was significantly associated with normal tension glaucoma. AA genotype of EDNRA:c.-231G > A polymorphism was associated with lower baseline intraocular pressure than in GG+GA genotype group. (PMID:16971893)
• Endothelin receptor A has a role in progression of prostate cancer (PMID:16984730)
• ET-1 mediates the increased vascular tone of extremely inactive legs of SCI individuals by increased activation of ET(A)-receptors (PMID:17122448)
• association of genetic variation with aortic pressure in patients with dilated cardiomyopathy (PMID:17198909)
• analysis of Endothelin-1, ETAR and ETBR expression in different histologic subtypes of renal cell carcinoma (PMID:17203161)
• Endogenous endothelin, predominantly via ET(A) receptor stimulation, contributes to basal constrictor tone and endothelial dysfunction, whereas ET(B) activation mediates vasodilation in human coronaries. (PMID:17353514)
• No association between the -231 G > A polymorphism in the EDNRA gene and preeclampsia as well as any correlation with the main clinical features of the disorder were found. (PMID:17437213)
• Hypoxia-induced breast carcinoma invasiveness was reduced by ETRA antagonist atrasentan. (PMID:17468950)
• neutrophils taken from healthy donors damage the endothelium by a mechanism dependent on ETs acting via ET(A) receptor, whereas neutrophils from acute pancreatitis patients cause more severe damage that is not dependent on ETs (PMID:17575543)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	ednraa	ENSDARG00000011876
danio_rerio	ednrab	ENSDARG00000062262
mus_musculus	Ednra	ENSMUSG00000031616
rattus_norvegicus	Ednra	ENSRNOG00000012721

Paralogs (15): NTSR1 (ENSG00000101188), BRS3 (ENSG00000102239), MLNR (ENSG00000102539), GHSR (ENSG00000121853), GRPR (ENSG00000126010), NMUR2 (ENSG00000132911), NMBR (ENSG00000135577), EDNRB (ENSG00000136160), NTSR2 (ENSG00000169006), GPR37L1 (ENSG00000170075), GPR37 (ENSG00000170775), NMUR1 (ENSG00000171596), GPR148 (ENSG00000173302), TRHR (ENSG00000174417), GPR39 (ENSG00000183840)

Protein

Protein identifiers

Endothelin-1 receptor — P25101 (reviewed: P25101)

Alternative names: Endothelin receptor type A

All UniProt accessions (1): P25101

UniProt curated annotations — full annotation on UniProt →

Function. Receptor for endothelin-1. Mediates its action by association with G proteins that activate a phosphatidylinositol-calcium second messenger system. The rank order of binding affinities for ET-A is: ET1 > ET2 » ET3.

Subunit / interactions. Interacts with HDAC7 and KAT5.

Subcellular location. Cell membrane.

Tissue specificity. Isoform 1, isoform 3 and isoform 4 are expressed in a variety of tissues, with highest levels in the aorta and cerebellum, followed by lung, atrium and cerebral cortex, lower levels in the placenta, kidney, adrenal gland, duodenum, colon, ventricle and liver but no expression in umbilical vein endothelial cells. Within the placenta, isoform 1, isoform 2, isoform 3 and isoform 4 are expressed in the villi and stem villi vessels.

Disease relevance. Mandibulofacial dysostosis with alopecia (MFDA) [MIM:616367] A form of mandibulofacial dysostosis, a disorder characterized by malar and mandibular hypoplasia, typically associated with abnormalities of the ears and eyelids. MFDA features include maxillary dysmorphism with dysplastic zygomatic arch, hypoplastic mandible, scalp alopecia, scant eyebrows and eyelashes, severe hypoplasia or aplasia of eyelids, small cupped dysplastic ears, conductive hearing loss, cleft palate, dental anomalies, micrognathia, and limited jaw mobility. The disease is caused by variants affecting the gene represented in this entry.

Similarity. Belongs to the G-protein coupled receptor 1 family. Endothelin receptor subfamily. EDNRA sub-subfamily.

Isoforms (5)

UniProt ID	Names	Canonical?
P25101-1	1	yes
P25101-2	2, Delta-3
P25101-3	3, Delta-4
P25101-4	4, Delta-3,4
P25101-5	5

RefSeq proteins (2): NP_001159527, NP_001948* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR000276	GPCR_Rhodpsn	Family
IPR000499	Endthln_rcpt	Family
IPR002175	ETA_rcpt	Family
IPR017452	GPCR_Rhodpsn_7TM	Domain
IPR051193	GPCR_endothelin_rcpt	Family

Pfam: PF00001

UniProt features (58 total): helix 12, sequence conflict 10, topological domain 8, transmembrane region 7, splice variant 6, sequence variant 3, strand 3, glycosylation site 2, signal peptide 1, chain 1, region of interest 1, compositionally biased region 1, modified residue 1, disulfide bond 1, turn 1

Structure

Experimental structures (PDB)

5 structures.

PDB	Method	Resolution (Å)
8HCQ	ELECTRON MICROSCOPY	3.01
8XVK	ELECTRON MICROSCOPY	3.21
8XVL	ELECTRON MICROSCOPY	3.22
8XVJ	ELECTRON MICROSCOPY	3.26
8XVI	ELECTRON MICROSCOPY	3.32

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-P25101-F1	74.45	0.45

Antibody-complex structures (SAbDab): 2 — 8HCQ, 8XVI

Functional residue map

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

Post-translational modifications (1): 425

Disulfide bonds (1): 158–239

Glycosylation sites (2): 29, 62

Function

Pathways and Gene Ontology

Reactome pathways

2 pathways

ID	Pathway
R-HSA-375276	Peptide ligand-binding receptors
R-HSA-416476	G alpha (q) signalling events

MSigDB gene sets: 609 (showing top): GOBP_CARDIAC_CHAMBER_DEVELOPMENT, GOBP_MORPHOGENESIS_OF_AN_EPITHELIUM, GOBP_ENDOTHELIAL_CELL_DEVELOPMENT, GOBP_PHENOL_CONTAINING_COMPOUND_METABOLIC_PROCESS, TURASHVILI_BREAST_LOBULAR_CARCINOMA_VS_DUCTAL_NORMAL_UP, GOBP_RESPONSE_TO_NITROGEN_COMPOUND, GOBP_CARBOHYDRATE_TRANSPORT, GOBP_EMBRYO_DEVELOPMENT_ENDING_IN_BIRTH_OR_EGG_HATCHING, GOBP_EPITHELIUM_DEVELOPMENT, GOBP_CELL_MIGRATION_INVOLVED_IN_HEART_DEVELOPMENT, WALLACE_PROSTATE_CANCER_RACE_UP, GOBP_RESPIRATORY_GASEOUS_EXCHANGE_BY_RESPIRATORY_SYSTEM, GOBP_MUSCLE_TISSUE_DEVELOPMENT, GOBP_REGULATION_OF_BLOOD_PRESSURE, GOBP_NEURON_PROJECTION_EXTENSION

GO Biological Process (85): mitotic cell cycle (GO:0000278), branching involved in blood vessel morphogenesis (GO:0001569), response to hypoxia (GO:0001666), in utero embryonic development (GO:0001701), blood vessel remodeling (GO:0001974), response to amphetamine (GO:0001975), regulation of heart rate (GO:0002027), glomerular filtration (GO:0003094), cardiac chamber formation (GO:0003207), left ventricular cardiac muscle tissue morphogenesis (GO:0003220), atrial cardiac muscle tissue development (GO:0003228), cardiac neural crest cell migration involved in outflow tract morphogenesis (GO:0003253), noradrenergic neuron differentiation (GO:0003357), intracellular calcium ion homeostasis (GO:0006874), smooth muscle contraction (GO:0006939), mitochondrion organization (GO:0007005), signal transduction (GO:0007165), G protein-coupled receptor signaling pathway (GO:0007186), activation of adenylate cyclase activity (GO:0007190), adenylate cyclase-inhibiting G protein-coupled receptor signaling pathway (GO:0007193), phospholipase C-activating G protein-coupled receptor signaling pathway (GO:0007200), positive regulation of cytosolic calcium ion concentration (GO:0007204), respiratory gaseous exchange by respiratory system (GO:0007585), regulation of blood pressure (GO:0008217), cell population proliferation (GO:0008283), response to wounding (GO:0009611), gene expression (GO:0010467), regulation of D-glucose transmembrane transport (GO:0010827), neural crest cell fate commitment (GO:0014034), artery smooth muscle contraction (GO:0014824), neuron remodeling (GO:0016322), heparin proteoglycan metabolic process (GO:0030202), thyroid gland development (GO:0030878), cellular response to oxidative stress (GO:0034599), embryonic heart tube development (GO:0035050), aorta development (GO:0035904), vasoconstriction (GO:0042310), norepinephrine metabolic process (GO:0042415), middle ear morphogenesis (GO:0042474), positive regulation of canonical NF-kappaB signal transduction (GO:0043123)

GO Molecular Function (4): phosphatidylinositol-4,5-bisphosphate phospholipase C activity (GO:0004435), endothelin receptor activity (GO:0004962), G protein-coupled receptor activity (GO:0004930), protein binding (GO:0005515)

GO Cellular Component (2): plasma membrane (GO:0005886), membrane (GO:0016020)

Reactome top-level categories

Rollup of top-2 pathways:

Category	Pathways
Class A/1 (Rhodopsin-like receptors)	1
GPCR downstream signalling	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cell cycle	1
mitotic nuclear division	1
angiogenesis	1
blood vessel morphogenesis	1
branching morphogenesis of an epithelial tube	1
response to stress	1
response to decreased oxygen levels	1
chordate embryonic development	1
tissue remodeling	1
response to amine	1
regulation of heart contraction	1
regulation of biological quality	1
renal filtration	1
cardiac chamber morphogenesis	1
anatomical structure formation involved in morphogenesis	1
cardiac left ventricle morphogenesis	1
ventricular cardiac muscle tissue morphogenesis	1
cardiac muscle tissue development	1
neural crest cell migration	1
outflow tract morphogenesis	1
cell migration involved in heart development	1
cardiac neural crest cell development involved in outflow tract morphogenesis	1
neuron differentiation	1
intracellular monoatomic cation homeostasis	1
calcium ion homeostasis	1
muscle contraction	1
organelle organization	1
cell communication	1
cellular process	1
signaling	1
regulation of cellular process	1
cellular response to stimulus	1
G protein-coupled receptor activity	1
signal transduction	1
positive regulation of adenylate cyclase activity	1
adenylate cyclase-modulating G protein-coupled receptor signaling pathway	1
adenylate cyclase inhibitor activity	1
C-type glycerophospholipase activity	1
G protein-coupled peptide receptor activity	1
endothelin receptor signaling pathway	1

Protein interactions and networks

STRING

1926 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
EDNRA	EDN1	P05305	999
EDNRA	EDN3	P14138	999
EDNRA	EDN2	P20800	999
EDNRA	EDNRB	P24530	943
EDNRA	ECE1	P42892	917
EDNRA	AGT	P01019	892
EDNRA	ECE2	P0DPD6	870
EDNRA	GNA12	Q03113	792
EDNRA	GNAQ	P50148	790
EDNRA	RNASE2	P10153	787
EDNRA	EEF1AKMT4-ECE2	P0DPD8	761
EDNRA	GNRH1	P01148	612
EDNRA	DSG1	Q02413	585
EDNRA	DGKH	Q86XP1	582
EDNRA	DPH3	Q96FX2	577

IntAct

17 interactions, top by confidence:

A	B	Type	Score
ARRB1	SRC	psi-mi:“MI:0914”(association)	0.620
ARRB2	SRC	psi-mi:“MI:0914”(association)	0.560
ARRB1	EDNRA	psi-mi:“MI:0915”(physical association)	0.500
ARRB2	EDNRA	psi-mi:“MI:0915”(physical association)	0.500
ARRB1	AXIN1	psi-mi:“MI:0914”(association)	0.500
SRC	EDNRA	psi-mi:“MI:0915”(physical association)	0.400
EDNRA	EDN1	psi-mi:“MI:0915”(physical association)	0.400
EDN1	EDNRA	psi-mi:“MI:0915”(physical association)	0.400
EDNRA	RAMP1	psi-mi:“MI:0915”(physical association)	0.400
RAMP1	EDNRA	psi-mi:“MI:0915”(physical association)	0.400
EDNRA	RAMP2	psi-mi:“MI:0915”(physical association)	0.400
RAMP3	EDNRA	psi-mi:“MI:0915”(physical association)	0.400
EDNRA	BCL2L11	psi-mi:“MI:0914”(association)	0.350
EDNRA	MGST3	psi-mi:“MI:0914”(association)	0.350
EDNRA	STIM1	psi-mi:“MI:0914”(association)	0.350

BioGRID (70): ARL5B (Affinity Capture-MS), SYNGR2 (Affinity Capture-MS), ND1 (Affinity Capture-MS), SLC5A3 (Affinity Capture-MS), EBP (Affinity Capture-MS), MTX1 (Affinity Capture-MS), LMBR1 (Affinity Capture-MS), COX1 (Affinity Capture-MS), PXMP2 (Affinity Capture-MS), MCL1 (Affinity Capture-MS), VKORC1L1 (Affinity Capture-MS), BCL2L11 (Affinity Capture-MS), BCL2L11 (Affinity Capture-MS), MCL1 (Affinity Capture-MS), MTX1 (Affinity Capture-MS)

ESM2 similar proteins: A5A4L1, B2ZI34, O08725, O54799, O62709, P14600, P21450, P21451, P21729, P24053, P24530, P25101, P25103, P26684, P28088, P28336, P30547, P30548, P30550, P32304, P32305, P33534, P33535, P34975, P35372, P35463, P41144, P41145, P42866, P47211, P48302, P52500, P56479, P56497, P79350, Q29010, Q2KIP6, Q5DUB3, Q5IS39, Q5IS84

Diamond homologs: A1ZAX0, B2ZI34, E7F7V7, F1MV99, F1R332, O08726, O08786, O43603, O54798, O54799, O62709, O88626, O88854, O97666, O97772, O97967, P05363, P08911, P08912, P21451, P21729, P22270, P24053, P24530, P25101, P26684, P28088, P28336, P28646, P30550, P30551, P30552, P30553, P30796, P30872, P30873, P30937, P30974, P31391, P32238

SIGNOR signaling

16 interactions.

A	Effect	B	Mechanism
EDNRA	up-regulates	GNAQ	binding
EDN1	up-regulates	EDNRA	binding
N-(3-methoxy-5-methyl-2-pyrazinyl)-2-[4-(1,3,4-oxadiazol-2-yl)phenyl]-3-pyridinesulfonamide	down-regulates	EDNRA	“chemical inhibition”
EDNRA	“up-regulates activity”	GNAS	binding
EDNRA	“up-regulates activity”	GNAL	binding
EDNRA	“up-regulates activity”	GNAI1	binding
EDNRA	“up-regulates activity”	GNAI3	binding
EDNRA	“up-regulates activity”	GNAO1	binding
EDNRA	“up-regulates activity”	GNAZ	binding
EDNRA	“up-regulates activity”	GNAQ	binding
EDNRA	“up-regulates activity”	GNA14	binding
Endothelin-1	“up-regulates activity”	EDNRA	“chemical activation”
hsa-miR-30a-3p	“down-regulates quantity by repression”	EDNRA	“post transcriptional regulation”
miRNA-30a-5p	“down-regulates quantity by destabilization”	EDNRA	“post transcriptional regulation”
EDNRA	up-regulates	GNA13	binding
sitaxentan	“down-regulates activity”	EDNRA	“chemical inhibition”

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 11 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
G alpha (s) signalling events	6	39.9×	5e-07
Diseases of signal transduction by growth factor receptors and second messengers	5	25.8×	3e-05

GO biological processes:

GO term	Partners	Fold	FDR
protein transport	5	19.9×	2e-04

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	2
Likely pathogenic	0
Uncertain significance	71
Likely benign	36
Benign	21

Top pathogenic / likely-pathogenic (2)

Variant ID	HGVS	Classification
190323	NM_001957.4(EDNRA):c.386A>T (p.Tyr129Phe)	Pathogenic
190324	NM_001957.4(EDNRA):c.907G>A (p.Glu303Lys)	Pathogenic

SpliceAI

1332 predictions. Top by Δscore:

Variant	Effect	Δscore
4:147532702:G:GT	donor_gain	1.0000
4:147535869:C:G	acceptor_gain	1.0000
4:147540375:A:AG	acceptor_gain	1.0000
4:147540376:G:GG	acceptor_gain	1.0000
4:147542476:A:AG	acceptor_gain	1.0000
4:147542477:G:GA	acceptor_gain	1.0000
4:147542477:GTC:G	acceptor_gain	1.0000
4:147519845:TTTCA:T	acceptor_loss	0.9900
4:147519846:TTCAG:T	acceptor_loss	0.9900
4:147519847:TCA:T	acceptor_loss	0.9900
4:147519848:CA:C	acceptor_loss	0.9900
4:147519849:AGC:A	acceptor_loss	0.9900
4:147519975:ACAGG:A	donor_loss	0.9900
4:147519976:CAG:C	donor_loss	0.9900
4:147519977:AGGT:A	donor_loss	0.9900
4:147519978:G:C	donor_loss	0.9900
4:147519979:G:T	donor_loss	0.9900
4:147519980:T:A	donor_loss	0.9900
4:147532503:CAGGT:C	acceptor_loss	0.9900
4:147532504:AGG:A	acceptor_loss	0.9900
4:147532505:G:A	acceptor_loss	0.9900
4:147535868:A:AG	acceptor_gain	0.9900
4:147535868:ACTTT:A	acceptor_gain	0.9900
4:147535872:T:TA	acceptor_gain	0.9900
4:147535875:A:AG	acceptor_gain	0.9900
4:147535876:G:GA	acceptor_gain	0.9900
4:147536025:AGCAG:A	donor_loss	0.9900
4:147536026:GCAG:G	donor_gain	0.9900
4:147536026:GCAGG:G	donor_loss	0.9900
4:147536027:CAG:C	donor_loss	0.9900

AlphaMissense

2873 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
4:147486042:A:C	S121R	1.000
4:147486044:T:A	S121R	1.000
4:147486044:T:G	S121R	1.000
4:147486046:T:C	L122P	1.000
4:147486054:G:A	G125R	1.000
4:147486054:G:C	G125R	1.000
4:147486055:G:A	G125E	1.000
4:147486058:A:C	D126A	1.000
4:147486058:A:G	D126G	1.000
4:147486058:A:T	D126V	1.000
4:147486061:T:C	L127P	1.000
4:147519938:G:A	G170R	1.000
4:147519938:G:C	G170R	1.000
4:147519968:A:C	S180R	1.000
4:147519970:T:A	S180R	1.000
4:147519970:T:G	S180R	1.000
4:147519978:G:T	R183M	1.000
4:147532585:T:A	W210R	1.000
4:147532585:T:C	W210R	1.000
4:147535929:C:A	P267H	1.000
4:147535929:C:G	P267R	1.000
4:147539859:T:C	F315L	1.000
4:147539861:T:A	F315L	1.000
4:147539861:T:G	F315L	1.000
4:147540439:C:A	P366H	1.000
4:147540448:T:A	L369Q	1.000
4:147540448:T:C	L369P	1.000
4:147540468:T:C	F376L	1.000
4:147540470:T:A	F376L	1.000
4:147540470:T:G	F376L	1.000

dbSNP variants (sampled 300 via entrez): RS1000106853 (4:147512554 C>A,T), RS1000194974 (4:147525295 T>C), RS1000226016 (4:147525952 A>C,G), RS1000290330 (4:147505792 G>A,T), RS10003447 (4:147526227 C>T), RS1000347580 (4:147539754 G>A,C), RS1000365603 (4:147492768 A>G), RS10003944 (4:147514209 G>A), RS1000410291 (4:147505169 A>G,T), RS1000422280 (4:147499160 A>C,G), RS1000525208 (4:147500324 G>T), RS1000573471 (4:147507483 T>C), RS1000684000 (4:147541360 T>C), RS1000708319 (4:147517872 A>G), RS1000750687 (4:147545233 A>C)

Disease associations

OMIM: gene MIM:131243 | disease phenotypes: MIM:157300, MIM:616367

GenCC curated gene-disease

Disease	Classification	Inheritance
mandibulofacial dysostosis with alopecia	Definitive	Autosomal dominant
cystic fibrosis	Supportive	Autosomal recessive

Mondo (3): migraine with or without aura, susceptibility to, 1 (MONDO:0008000), mandibulofacial dysostosis with alopecia (MONDO:0014608), cystic fibrosis (MONDO:0009061)

Orphanet (1): Mandibulofacial dysostosis with alopecia (Orphanet:443995)

HPO phenotypes

67 total (30 of 67 shown, HPO-id order):

HPO	Term
HP:0000006	Autosomal dominant inheritance
HP:0000072	Hydroureter
HP:0000162	Glossoptosis
HP:0000175	Cleft palate
HP:0000211	Trismus
HP:0000232	Everted lower lip vermilion
HP:0000246	Sinusitis
HP:0000324	Facial asymmetry
HP:0000327	Hypoplasia of the maxilla
HP:0000347	Micrognathia
HP:0000365	Hearing impairment
HP:0000369	Low-set ears
HP:0000378	Cupped ear
HP:0000384	Preauricular skin tag
HP:0000402	Stenosis of the external auditory canal
HP:0000405	Conductive hearing impairment
HP:0000411	Protruding ear
HP:0000431	Wide nasal bridge
HP:0000613	Photophobia
HP:0000652	Lower eyelid coloboma
HP:0000653	Sparse eyelashes
HP:0000678	Dental crowding
HP:0000680	Delayed eruption of primary teeth
HP:0000716	Depression
HP:0000739	Anxiety
HP:0000787	Nephrolithiasis
HP:0000938	Osteopenia
HP:0000939	Osteoporosis
HP:0001392	Abnormality of the liver
HP:0001394	Cirrhosis

GWAS associations

36 associations (top):

Study	Trait	p-value
GCST001231_1	Carotid intima media thickness	7.000000e-07
GCST001231_10	Carotid intima media thickness	7.000000e-12
GCST001388_1	Intracranial aneurysm	1.000000e-08
GCST001713_12	Dental caries	2.000000e-07
GCST001762_181	Obesity-related traits	8.000000e-06
GCST002289_21	Coronary artery disease	1.000000e-06
GCST002290_9	Coronary artery disease or large artery stroke	2.000000e-08
GCST002935_12	Lead levels	2.000000e-06
GCST003116_33	Coronary artery disease	9.000000e-10
GCST003117_25	Myocardial infarction	4.000000e-07
GCST003133_12	Plasma clusterin levels	6.000000e-06
GCST003154_1	Peripheral artery disease	5.000000e-09
GCST003818_77	Resting heart rate	2.000000e-14
GCST004607_228	Plateletcrit	3.000000e-09
GCST004787_28	Coronary artery disease (myocardial infarction, percutaneous transluminal coronary angioplasty, coronary artery bypass grafting, angina or chromic ischemic heart disease)	5.000000e-10
GCST005195_12	Coronary artery disease	5.000000e-24
GCST005196_125	Coronary artery disease	1.000000e-23
GCST005434_32	Pancreatic cancer	4.000000e-06
GCST007017_10	Serum bilirubin levels x Mediterranean diet adherence interaction in metabolic syndrome	6.000000e-06
GCST007096_83	Pulse pressure	4.000000e-16
GCST007097_126	Pulse pressure	7.000000e-06
GCST007097_127	Pulse pressure	2.000000e-06
GCST007099_72	Systolic blood pressure	2.000000e-09
GCST007235_2	Pancreatic ductal adenocarcinoma	1.000000e-07
GCST007435_1	Carotid plaque	4.000000e-10
GCST008474_1	Peripheral artery disease	2.000000e-09
GCST010479_70	Coronary artery disease	2.000000e-12
GCST010866_26	Coronary artery disease	1.000000e-38
GCST010867_30	Coronary artery disease	8.000000e-11
GCST010867_58	Coronary artery disease	1.000000e-19

EFO canonical traits (12, from GWAS)

EFO ID	Trait name
EFO:0004578	homocysteine measurement
EFO:0007656	plasma clusterin measurement
EFO:0007985	platelet crit
EFO:0004570	bilirubin measurement
EFO:0008111	diet measurement
EFO:0005763	pulse pressure measurement
EFO:0006335	systolic blood pressure
EFO:0009783	carotid atherosclerosis
EFO:0005670	smoking initiation
EFO:0007984	platelet component distribution width
EFO:0004309	platelet count
EFO:0004305	erythrocyte count

MeSH disease descriptors (1)

Descriptor	Name	Tree numbers
D003550	Cystic Fibrosis	C06.689.202; C08.381.187; C16.320.190; C16.614.213

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (2): CHEMBL2096678 (PROTEIN FAMILY), CHEMBL252 (SINGLE PROTEIN)

Molecules with ChEMBL bioactivity

31 molecules (phase ≥1), by development phase (incl. off-target/promiscuous compounds). Patent mentions across the top 20 by phase: 497,798 (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
CHEMBL1111	AMBRISENTAN	4	7,009
CHEMBL1201469	GRAMICIDIN	4
CHEMBL1401	NITAZOXANIDE	4	9,504
CHEMBL1513	IRBESARTAN	4	31,667
CHEMBL184	ACYCLOVIR	4	97,488
CHEMBL2103873	MACITENTAN	4	1,372
CHEMBL2165326	APROCITENTAN	4	165
CHEMBL282724	SITAXENTAN	4	639
CHEMBL41	FLUOXETINE	4	62,368
CHEMBL437	SULFATHIAZOLE	4	7,148
CHEMBL453	SULFISOXAZOLE	4	21,363
CHEMBL535	SUNITINIB	4	79,020
CHEMBL539423	SPARSENTAN	4	354
CHEMBL595	PIOGLITAZONE	4	57,130
CHEMBL599	MELOXICAM	4	50,000
CHEMBL633	AMIODARONE	4	29,704
CHEMBL826	ENOXACIN	4	25,001
CHEMBL957	BOSENTAN	4	16,499
CHEMBL109648	CLAZOSENTAN	3	244
CHEMBL1628688	ZIBOTENTAN	3	1,123
CHEMBL23261	DARUSENTAN	3
CHEMBL3989834	AVOSENTAN	3
CHEMBL488025	EXISULIND	3
CHEMBL61780	TEZOSENTAN	3
CHEMBL9194	ATRASENTAN	3
CHEMBL2110610	FANDOSENTAN	2
CHEMBL314691	BQ-123	2
CHEMBL316735	FELOPRENTAN	2
CHEMBL383581	EDONENTAN	2
CHEMBL431651	ENRASENTAN	2

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

PharmGKB variants

1 variants.

Variant	Genes	Level	Score	#Clin annots	Drugs
rs5333	EDNRA		0.00	0

GtoPdb / IUPHAR curated pharmacology

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

Target class: gpcr — Endothelin receptors

Most potent curated ligand interactions (33 total), top 25:

Ligand	Action	Affinity	Parameter
edonentan	Antagonist	11.0	pKi
[125I]ET-1 (human, mouse, rat)	Full agonist	10.5	pKd
[125I]PD164333	Antagonist	9.8	pKd
[125I]sarafotoxin S6b	Full agonist	9.8	pKd
clazosentan	Antagonist	9.5	pA2
SB209670	Antagonist	9.4	pKB
macitentan	Antagonist	9.3	pIC50
[3H]S0139	Antagonist	9.2	pKd
atrasentan	Antagonist	9.2	pA2
PD-156707	Antagonist	9.2	pA2
[125I]PD151242	Antagonist	9.1	pKd
[125I]ET-2 (human)	Full agonist	9.1	pKd
SB234551	Antagonist	9.0	pIC50
PD164333	Antagonist	9.0	pKd
darusentan	Antagonist	8.9	pKi
BMS-193884	Antagonist	8.85	pKi
diosuxentan	Antagonist	8.85	pIC50
[3H]BQ123	Antagonist	8.5	pKd
endothelin-1	Full agonist	8.5	pIC50
TAK 044	Antagonist	8.4	pA2
zibotentan	Antagonist	8.3	pIC50
endothelin-2	Full agonist	8.2	pIC50
[18F]ET-1 (human, mouse, rat)	Full agonist	8.2	pKd
sarafotoxin S6b	Full agonist	8.1	pIC50
sparsentan	Antagonist	8.03	pKi

Binding affinities (BindingDB)

7 measured of 8 human assays (9 total across all organisms); most potent 7 below. Values come from heterogeneous assays and are not directly comparable.

Ligand	Measure	Value
J-104132	KI	0.1 nM
(1S,2R,3S)-1-Benzo[1,3]dioxol-5-yl-3-(2-carboxymethoxy-4-methoxy-phenyl)-5-propoxy-indan-2-carboxylic acid	KI	21 nM
Enrasentan	KI	150 nM
SB-234551	KI	500 nM
SR 147778	KI	1000 nM
PD156707	KI	1420 nM
(S)-3-[(S)-2-((S)-2-Acetylamino-3-adamantan-1-yl-propionylamino)-4-methyl-pentanoylamino]-N-((1S,2S)-1-{(1S,2S)-1-[(S)-1-carboxy-2-(1H-indol-3-yl)-ethylcarbamoyl]-2-methyl-butylcarbamoyl}-2-methyl-butyl)-succinamic acid	IC50	3200 nM

ChEMBL bioactivities

1780 potent at pChembl≥5 of 1875 total, top 50 by pChembl (potency: 10 = 0.1 nM, 6 = 1 µM).

pChembl	Type	Value	Unit	Molecule
11.00	Ki	0.01	nM	CHEMBL274489
11.00	Ki	0.01	nM	CHEMBL277447
11.00	Ki	0.01	nM	CHEMBL440780
10.96	IC50	0.011	nM	CHEMBL411925
10.96	IC50	0.011	nM	CHEMBL289634
10.96	IC50	0.011	nM	CHEMBL286621
10.85	IC50	0.014	nM	CHEMBL287683
10.85	IC50	0.014	nM	CHEMBL432856
10.82	Ki	0.015	nM	CHEMBL1627022
10.70	IC50	0.02	nM	CHEMBL294291
10.70	IC50	0.02	nM	CHEMBL60058
10.70	Ki	0.02	nM	CHEMBL29793
10.70	Ki	0.02	nM	CHEMBL281659
10.70	Ki	0.02	nM	CHEMBL285832
10.66	IC50	0.022	nM	CHEMBL35984
10.64	IC50	0.023	nM	CHEMBL3775234
10.64	IC50	0.023	nM	CHEMBL4128926
10.62	IC50	0.024	nM	CHEMBL287683
10.60	Ki	0.025	nM	ENDOTHELIN
10.60	IC50	0.025	nM	CHEMBL289634
10.57	IC50	0.027	nM	CHEMBL3775234
10.57	IC50	0.027	nM	CHEMBL37427
10.52	IC50	0.03	nM	CHEMBL293830
10.52	IC50	0.03	nM	CHEMBL304460
10.52	IC50	0.03	nM	CHEMBL60367
10.52	Ki	0.03	nM	CHEMBL27855
10.51	IC50	0.031	nM	CHEMBL287622
10.47	IC50	0.034	nM	ATRASENTAN
10.47	Ki	0.034	nM	ATRASENTAN
10.47	Ki	0.034	nM	CHEMBL3775234
10.44	IC50	0.036	nM	CHEMBL289216
10.41	Ki	0.039	nM	CHEMBL128818
10.40	IC50	0.04	nM	CHEMBL416324
10.40	IC50	0.04	nM	CHEMBL19950
10.40	IC50	0.04	nM	CHEMBL60149
10.40	Ki	0.04	nM	CHEMBL2113316
10.40	Ki	0.04	nM	CHEMBL282359
10.40	Ki	0.04	nM	CHEMBL28863
10.39	IC50	0.041	nM	CHEMBL288247
10.36	IC50	0.044	nM	CHEMBL323464
10.35	IC50	0.045	nM	CHEMBL411925
10.35	Ki	0.045	nM	CHEMBL30405
10.33	IC50	0.047	nM	ENDOTHELIN
10.33	IC50	0.047	nM	CHEMBL36627
10.32	IC50	0.048	nM	CHEMBL286621
10.30	IC50	0.05	nM	ENDOTHELIN
10.30	IC50	0.05	nM	CHEMBL291592
10.30	IC50	0.05	nM	CHEMBL62159
10.30	Ki	0.05	nM	CHEMBL281549
10.30	IC50	0.05	nM	CHEMBL291058

PubChem BioAssay actives

1360 with measured affinity, of 3178 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
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N,3,3-trimethylbutanamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-[2-[(E)-N-hydroxy-C-methylcarbonimidoyl]-4,6-dimethylphenyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-(2,4-dimethyl-6-methylsulfonylphenyl)thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
(2R,3R,4S)-4-(1,3-benzodioxol-5-yl)-1-[2-[butylsulfonyl(propyl)amino]ethyl]-2-(3-fluoro-4-methoxyphenyl)pyrrolidine-3-carboxylic acid	68307: Binding assay performed using human Endothelin A receptor (hETA) expressed in chinese hamster ovary cells(CHO).	ic50	<0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-[2-(cyclopropanecarbonyl)-4,6-dimethylphenyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
N-(2-acetyl-3,4,6-trimethylphenyl)-3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-(3,4,6-trimethyl-2-propanoylphenyl)thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
N-(3,4-dimethyl-1,2-oxazol-5-yl)-2-[2-[(N-methylanilino)methyl]-4-(1,3-oxazol-2-yl)phenyl]benzenesulfonamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-(3,4-dimethyl-1,2-oxazol-5-yl)-2-[2-[(4,4-dimethyl-2-oxopyrrolidin-1-yl)methyl]-4-(1,3-oxazol-2-yl)phenyl]benzenesulfonamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
3,5-dichloro-N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N-methylbenzamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N-methyl-2-[4-(trifluoromethyl)phenyl]acetamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N-methyl-3-phenylpropanamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N,2-dimethylpropanamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N-methyl-2-phenylacetamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-(3,4-dimethyl-1,2-oxazol-5-yl)-2-[2-[(3-methyl-2-oxoimidazolidin-1-yl)methyl]-4-(1,3-oxazol-2-yl)phenyl]benzenesulfonamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-(3,4-dimethyl-1,2-oxazol-5-yl)-2-[4-(1,3-oxazol-2-yl)-2-[[3-(trifluoromethyl)pyrazol-1-yl]methyl]phenyl]benzenesulfonamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N,1-dimethylindole-2-carboxamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-(3,4-dimethyl-1,2-oxazol-5-yl)-2-[2-[(3,3-dimethyl-2-oxopyrrolidin-1-yl)methyl]-4-(1,3-oxazol-2-yl)phenyl]benzenesulfonamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N,2,2-trimethylpropanamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-[2-[(E)-N-methoxy-C-methylcarbonimidoyl]-4,6-dimethylphenyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-N-methylbenzamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
(2R,3R,4S)-4-(1,3-benzodioxol-5-yl)-1-[2-(dibutylamino)-2-oxoethyl]-2-(4-methoxyphenyl)pyrrolidine-3-carboxylic acid	1631613: Displacement of [125I]ET-1 from human ET-A receptor expressed in CHO cell membrane	ki	<0.0001	uM
(3S)-3-[[(2S)-2-[[(2S)-2-[[(1R,4S,7S,10S,13S,16S,19S,22S,25R,28S,31R,36R,39S,42S,45S)-31-amino-7-(4-aminobutyl)-39-benzyl-4-(2-carboxyethyl)-10-(carboxymethyl)-19,22,28-tris(hydroxymethyl)-42-[(4-hydroxyphenyl)methyl]-16-(2-methylpropyl)-13-(2-methylsulfanylethyl)-3,6,9,12,15,18,21,24,27,30,38,41,44,47-tetradecaoxo-45-propan-2-yl-33,34,49,50-tetrathia-2,5,8,11,14,17,20,23,26,29,37,40,43,46-tetradecazabicyclo[23.22.4]henpentacontane-36-carbonyl]amino]-3-(1H-imidazol-5-yl)propanoyl]amino]-4-methylpentanoyl]amino]-4-[[(2S,3S)-1-[[(2S,3S)-1-[[(1S)-1-carboxy-2-(1H-indol-3-yl)ethyl]amino]-3-methyl-1-oxopentan-2-yl]amino]-3-methyl-1-oxopentan-2-yl]amino]-4-oxobutanoic acid	1285629: Displacement of [125I]Endothelin-1 from human recombinant Endothelin-1 receptor expressed in CHO cells	ic50	<0.0001	uM
methyl 2-[[3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]thiophene-2-carbonyl]amino]-5-methylbenzoate	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
sodium [6-[2-(5-bromopyrimidin-2-yl)oxyethoxy]-5-(4-methylphenyl)pyrimidin-4-yl]-[4-(1-hydroxy-2-methylpropan-2-yl)phenyl]sulfonylazanide	68472: Ability to inhibit [125I]ET1 binding to human cloned endothelin A receptor expressed on CHO cells	ki	<0.0001	uM
N-[[2-[2-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]methyl]-3,3-dimethylbutanamide	66344: Inhibitory concentration against Endothelin A receptor	ki	<0.0001	uM
N-[2-[2-[(4,5-dimethyl-1,2-oxazol-3-yl)sulfamoyl]phenyl]-5-(1,3-oxazol-2-yl)phenyl]-N,3,3-trimethylbutanamide	1978791: Antagonist activity at human ETA receptor expressed in CHO cells assessed as inhibition constant	ki	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-(1-hydroxypropan-2-yloxy)-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-(3-hydroxy-2-methylpropyl)-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-(3-hydroxypropyl)-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-(2-hydroxyethyl)-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-7-[2-[[acetyl(methyl)amino]methyl]-4-methoxyphenyl]-5-(1,3-benzodioxol-5-yl)-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-[2-(hydroxymethyl)butyl]-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-[3-(dimethylamino)-2-methyl-3-oxopropyl]-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-(2-hydroxy-2-methylpropyl)-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-(2,4-dimethoxyphenyl)-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[4-methoxy-2-(3-methoxy-2-methylpropyl)phenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(5S,6R,7R)-5-(1,3-benzodioxol-5-yl)-7-[2-(2,3-dihydroxypropyl)-4-methoxyphenyl]-2-(propan-2-ylamino)-6,7-dihydro-5H-cyclopenta[b]pyridine-6-carboxylic acid	68325: Inhibitory activity against [125I]ET1 binding to human endothelin A receptor	ic50	<0.0001	uM
(3S)-3-[[(2S)-2-[[(2S)-2-[[(1S,4S,7S,10R,13S,16R,19S,22R,25R,28R,31S,36R,39S,42R,45S)-31-amino-7-(4-aminobutyl)-39-benzyl-4-(2-carboxyethyl)-10-(carboxymethyl)-19,22,28-tris(hydroxymethyl)-42-[(4-hydroxyphenyl)methyl]-16-(2-methylpropyl)-13-(2-methylsulfanylethyl)-3,6,9,12,15,18,21,24,27,30,38,41,44,47-tetradecaoxo-45-propan-2-yl-33,34,49,50-tetrathia-2,5,8,11,14,17,20,23,26,29,37,40,43,46-tetradecazabicyclo[23.22.4]henpentacontane-36-carbonyl]amino]-3-(1H-imidazol-4-yl)propanoyl]amino]-4-methylpentanoyl]amino]-4-[[(2S,3S)-1-[[(2S,3S)-1-[[(1S)-1-carboxy-2-(1H-indol-3-yl)ethyl]amino]-3-methyl-1-oxopentan-2-yl]amino]-3-methyl-1-oxopentan-2-yl]amino]-4-oxobutanoic acid	468184: Inhibition of ETA receptor in human SK-N-MC cells after 60 mins by scintillation counting	ic50	<0.0001	uM
3-[[5-[4-(tert-butylsulfamoyl)naphthalen-1-yl]-4-(cyclohexylmethyl)-1,3-thiazole-2-carbonyl]amino]cyclobutane-1-carboxylic acid	1494742: Displacement of [125I]-endothelin-1 from human recombinant ETA receptor after 120 mins by scintillation counting analysis	ic50	<0.0001	uM
N-(2-acetyl-4,6-dimethylphenyl)-3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	<0.0001	uM
(2R,3R,4S)-1-[2-(dibutylamino)-2-oxoethyl]-4-(2,3-dihydro-1-benzofuran-5-yl)-2-(3-fluoro-4-methylphenyl)pyrrolidine-3-carboxylic acid	68475: Binding affinity against human Endothelin A receptor	ki	<0.0001	uM
N-[6-[2-(5-bromopyrimidin-2-yl)oxyethoxy]-5-(4-methylphenyl)pyrimidin-4-yl]-4-tert-butylbenzenesulfonamide	68472: Ability to inhibit [125I]ET1 binding to human cloned endothelin A receptor expressed on CHO cells	ki	<0.0001	uM
N-(4,5-dimethyl-1,2-oxazol-3-yl)-2-[2-[(3,3-dimethyl-2-oxopyrrolidin-1-yl)methyl]-4-(1,3-oxazol-2-yl)phenyl]benzenesulfonamide	68487: Inhibitory activity against human endothelin A receptor expressed in CHO cells	ki	<0.0001	uM
N-(2-cyano-3,4,6-trimethylphenyl)-3-[(3,4-dimethyl-1,2-oxazol-5-yl)sulfamoyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	0.0001	uM
N-(2-chloro-4,6-dimethylphenyl)-3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-(2,4-dimethyl-6-propylsulfonylphenyl)thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	0.0001	uM
N-(4-chloro-3-methyl-1,2-oxazol-5-yl)-2-[2-(3-hydroxy-2,4,6-trimethylphenyl)acetyl]thiophene-3-sulfonamide	68332: Inhibitory concentration was determined against selective Endothelin A receptor	ic50	0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-(2,4-dimethyl-6-propan-2-ylsulfonylphenyl)thiophene-2-carboxamide	66198: Displacement of [125I]ET-1 from Endothelin A receptor	ic50	0.0001	uM
3-[(4-chloro-3-methyl-1,2-oxazol-5-yl)sulfamoyl]-N-[2,4-dimethyl-6-(2-methylpropanoyl)phenyl]thiophene-2-carboxamide	68332: Inhibitory concentration was determined against selective Endothelin A receptor	ic50	0.0001	uM

CTD chemical–gene interactions

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

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	decreases expression, affects cotreatment, increases expression, affects expression	8
trichostatin A	affects cotreatment, increases expression	3
Atrasentan	affects binding, decreases activity	3
Aflatoxin B1	decreases methylation, increases expression, increases methylation	3
sodium arsenite	affects splicing, affects reaction, decreases expression	2
cyclo(Trp-Asp-Pro-Val-Leu)	affects binding, decreases activity	2
darusentan	affects binding, decreases activity	2
belinostat	affects cotreatment, increases expression	2
Temozolomide	affects response to substance, decreases expression	2
Fulvestrant	decreases expression, increases methylation, affects cotreatment	2
Vorinostat	affects cotreatment, increases expression	2
Panobinostat	affects cotreatment, increases expression	2
Benzo(a)pyrene	decreases expression, increases methylation	2
Doxorubicin	decreases expression	2
Phenylmercuric Acetate	affects cotreatment, increases expression	2
Silicon Dioxide	decreases expression	2
Tamoxifen	affects cotreatment, decreases expression, increases expression, decreases reaction	2
Tobacco Smoke Pollution	decreases expression	2
Tretinoin	decreases expression, increases expression	2
testosterone enanthate	affects expression	1
methylmercuric chloride	decreases expression	1
alpha-pinene	affects cotreatment, increases oxidation, increases abundance	1
methylselenic acid	increases expression	1
steviol	increases expression	1
stevioside	increases expression	1
sulforaphane	decreases expression	1
rebaudioside A	increases expression	1
butylbenzyl phthalate	increases expression	1
aflatoxin B2	increases methylation	1
methacrylaldehyde	increases oxidation, increases abundance, affects cotreatment	1

ChEMBL screening assays

418 unique, capped per target: 342 binding, 73 functional, 2 toxicity, 1 admet

Representative assays (with source publication via chembl_document):

Assay ID	Type	Description	Source paper
CHEMBL671535	Binding	Ratio of endothelin A receptor and endothelin B receptor.	Pyrrolidine-3-carboxylic acids as endothelin antagonists. 2. Sulfonamide-based ETA/ETB mixed antagonists. — J Med Chem
CHEMBL676577	Functional	Compound was evaluated for the Inhibition of the contractile response of Endothelin A receptor in rabbit thoracic aorta	Endothelin: a new challenge. — J Med Chem
CHEMBL4810247	ADMET	Inhibition of human ETA receptor at 0.1 to 1 uM	Discovery of Pemigatinib: A Potent and Selective Fibroblast Growth Factor Receptor (FGFR) Inhibitor. — J Med Chem

Cellosaurus cell lines

8 cell lines: 4 cancer cell line, 2 transformed cell line, 2 spontaneously immortalized cell line

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

Cellosaurus	Name	Category	Sex
CVCL_D1W6	Abcam A-549 EDNRA KO	Cancer cell line	Male
CVCL_D2AJ	Abcam HCT 116 EDNRA KO	Cancer cell line	Male
CVCL_D8KN	Ubigene HCT 116 EDNRA KO	Cancer cell line	Male
CVCL_D9DY	Ubigene HEK293 EDNRA KO	Transformed cell line	Female
CVCL_H426	CHO-K1/EDNRA	Spontaneously immortalized cell line	Female
CVCL_KB34	GeneBLAzer EDNRA-NFAT-bla HEK 293T	Transformed cell line	Female
CVCL_KW99	PathHunter CHO-K1 EDNRA beta-arrestin	Spontaneously immortalized cell line	Female
CVCL_YK43	U2OS EDNRA HiTSeeker	Cancer cell line	Female

Clinical trials (associated diseases)

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

Trial	Phase	Status	Title
NCT00157690	PHASE4	COMPLETED	Study of Alendronate to Prevent and Treat Osteoporosis in Cystic Fibrosis Patients
NCT00208078	PHASE4	TERMINATED	Effect of Non-Invasive Ventilation in Cystic Fibrosis Patient With Chronic Respiratory Failure.
NCT00244270	PHASE4	COMPLETED	Cystic Fibrosis and Totally Implantable Vascular Access Devices
NCT00333385	PHASE4	TERMINATED	Continuous Versus Short Infusions of Ceftazidime in Cystic Fibrosis
NCT00411736	PHASE4	COMPLETED	Scandinavian Cystic Fibrosis Azithromycin Study
NCT00418470	PHASE4	TERMINATED	Prolonging the Duration of Peripheral Venous Catheters in Cystic Fibrosis People
NCT00431964	PHASE4	COMPLETED	Effect of Azithromycin on Lung Function in 6-18 Year-olds With Cystic Fibrosis (CF) Not Infected With P. Aeruginosa
NCT00434278	PHASE4	TERMINATED	A Trial of Pulmozyme Withdrawal on Exercise Tolerance in Cystic Fibrosis Subjects With Severe Lung Disease (TOPIC)
NCT00483769	PHASE4	COMPLETED	One Year Glargine Treatment in CFRD Children and Adolescents
NCT00528190	PHASE4	COMPLETED	Treatment of Aspergillus Fumigatus (a Fungal Infection) in Patients With Cystic Fibrosis
NCT00557089	PHASE4	COMPLETED	The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis
NCT00572975	PHASE4	COMPLETED	Malabsorption Blood Test:Toward a Novel Approach to Quantify Steatorrhea
NCT00680316	PHASE4	TERMINATED	A Study of Pulmozyme® (Dornase Alpha) in 3- to 5-Year-Old Patients With Cystic Fibrosis
NCT00685035	PHASE4	COMPLETED	Comparison of Airway Clearance Therapy in Cystic Fibrosis Using the Same VEST Therapy Device But With Different Settings
NCT00744250	PHASE4	TERMINATED	Intraduodenal Aspiration Study to Assess the Bioavailability of Oral Pancrecarb® Compared to Placebo Control
NCT00787917	PHASE4	TERMINATED	An Exploratory Study to Assess Multiple Doses of Omalizumab in Patients With Cystic Fibrosis Complicated by Acute Bronchopulmonary Aspergillosis (ABPA)
NCT00843817	PHASE4	COMPLETED	RhDNase and Biodistribution of PMN Serine Proteases in Cystic Fibrosis Sputum
NCT00890370	PHASE4	COMPLETED	Should Any One Airway Clearance Technique be Recommended for People With Cystic Fibrosis?
NCT00996424	PHASE4	TERMINATED	The Effect of Inhaled N-Acetylcysteine Compared to Normal Saline on Sputum Rheology and Lung Function
NCT01044719	PHASE4	UNKNOWN	Duration of Antibiotics in Infective Exacerbations of Cystic Fibrosis
NCT01100606	PHASE4	COMPLETED	A Study to Evaluate the Mode of Administration and Safety of EUR-1008 (APT-1008) in Infants 1 to 12 Months of Age
NCT01131507	PHASE4	COMPLETED	PR-018: An Open-Label, Safety Extension of Study PR-011
NCT01207245	PHASE4	COMPLETED	Circadian Rhythm In Tobramycin Elimination In Cystic Fibrosis
NCT01323101	PHASE4	COMPLETED	Doxycycline Effects on Inflammation in Cystic Fibrosis
NCT01327703	PHASE4	COMPLETED	Control of Steatorrhea in Participants With Cystic Fibrosis and Exocrine Pancreatic Insufficiency
NCT01377792	PHASE4	COMPLETED	Study of Long-term Treatment With Hypertonic Saline in Patients With Cystic Fibrosis
NCT01400750	PHASE4	COMPLETED	Comparison of 2 Treatment Regimens for Eradication of P Aeruginosa Infection in Children With Cystic Fibrosis
NCT01429259	PHASE4	COMPLETED	Population Pharmacokinetics of Prolonged Infusion Meropenem in Cystic Fibrosis (CF) Children
NCT01608555	PHASE4	COMPLETED	Tobramycin 300 mg Once-a-day (o.d.) Aerosol in Adults With Cystic Fibrosis
NCT01667094	PHASE4	UNKNOWN	A Study Comparing Continuous Infusion Antibiotics to Standard Treatment for Lung Infections in Cystic Fibrosis
NCT01694069	PHASE4	TERMINATED	Continuous Infusion Piperacillin-tazobactam for the Treatment of Cystic Fibrosis
NCT01702415	PHASE4	WITHDRAWN	Zoledronic Acid in Cystic Fibrosis
NCT01712334	PHASE4	COMPLETED	A Study of the Comparable Efficacy and Safety of Pulmozyme (Dornase Alfa) Delivered by the eRapid Nebulizer System in Patients With Cystic Fibrosis
NCT01737983	PHASE4	COMPLETED	Effect of Lactobacillus Reuteri in Cystic Fibrosis
NCT01844778	PHASE4	COMPLETED	Ease of Use and Microbial Contamination of Tobramycin Inhalation Powder (TIP) Versus Nebulised Tobramycin Inhalation Solution (TIS) and Nebulised Colistimethate (COLI)
NCT01880346	PHASE4	COMPLETED	Comparison of Absorption of Vitamin D in Cystic Fibrosis
NCT01882400	PHASE4	COMPLETED	Assessment of Response to Treatment of Osteoporosis With Oral Bisphosphonates in Patients With Muscular Dystrophy
NCT01937325	PHASE4	UNKNOWN	CPET in CF Patients With One G551D Mutation Taking VX770
NCT02015663	PHASE4	TERMINATED	Tobramycin Inhalation Powder (TIP) Administered Once Daily Continuously Versus TIP Administered BID in 28 Day on / 28 Day Off Cycles
NCT02048592	PHASE4	UNKNOWN	Impact of Immunonutrition on the Patients With Cystic Fibrosis