EDNRA endothelin receptor type A
ENSG00000151617 · protein coding · Open Targets profile
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.
Each band is a group of drugs. It runs from the kind of drug on the left to the furthest stage that drug has reached in any disease on the right. Thicker bands mean more drugs.
Approved bands are highlighted. Withdrawn means the drug was pulled from the market or from development. A combined phase counts as the later one (Phase 1/2 is shown as Phase 2). Hover a band for the drug names. Source: Open Targets Platform, fetched live.
Each row is a cancer, strongest overall evidence first. The two dots show how strong the inherited (germline) and tumour-mutation (somatic) evidence is; the line between them is the gap.
Scores run from 0 (no evidence) to 1 (strongest). A missing dot at 0 means Open Targets holds no evidence of that kind. Source: Open Targets Platform, fetched live.
Tractability
- Small molecule
- Approved Drug, Structure with Ligand, High-Quality Ligand, Druggable Family
- Antibody
- GO CC high conf, UniProt loc med conf, UniProt SigP or TMHMM
- PROTAC
- Small Molecule Binder
- Other modalities
- Advanced Clinical
gnomAD loss of function constraint: o/e 0.265639990568161 (upper bound 0.43970000743865967). Low values mean loss of function is poorly tolerated, a safety flag for inhibition.
Who runs trials of these drugs
Principal investigators and study chairs on 169 registry trials, updated in the last three years, of the most advanced drugs against EDNRA (aprocitentan, clazosentan, sparsentan, macitentan, ambrisentan, bosentan, darusentan, zibotentan, avosentan, tezosentan, BQ-123, enrasentan), ranked by Phase 2+ trials. Live from ClinicalTrials.gov.
- Radko KomersTravere Therapeutics, Inc. · 5 of 5 trials Phase 2+
- Andjela Kusic-PajicActelion Pharmaceuticals Australia Pty. Ltd · 3 of 3 trials Phase 2+
- Loïc PerchenetActelion · 3 of 3 trials Phase 2+
- Pär I JohanssonCopenhagen University Hospital, Rigshospitalet · 3 of 3 trials Phase 2+
- Isabelle LeconteActelion · 2 of 2 trials Phase 2+
- James SeiboldRobert Wood Johnson Medical School, New Brunswick, NJ, USA · 2 of 2 trials Phase 2+
- Nicholas S HillTufts Medical Center · 2 of 2 trials Phase 2+
- Neeraj DhaunUniversity of Edinburgh · 1 of 2 trials Phase 2+
- Aaron B WaxmanBrigham and Women's Hospital · 1 of 1 trials Phase 2+
- Alexander RothmanUniversity of Sheffield · 1 of 1 trials Phase 2+
- Alice BEREZNEAssistance Publique - Hôpitaux de Paris · 1 of 1 trials Phase 2+
- Amadeo MarcosUniversity of Pittsburgh Medical Center · 1 of 1 trials Phase 2+
- Anders PernerCopenhagen University Hospital, Intensive care Unit 4131 · 1 of 1 trials Phase 2+
- André DenaultMontreal Heart Institute · 1 of 1 trials Phase 2+
- Andrea LauerActelion Pharmaceuticals US, Inc. · 1 of 1 trials Phase 2+
Registry trials of aprocitentan, clazosentan, sparsentan, macitentan, ambrisentan, bosentan, darusentan, zibotentan, avosentan, tezosentan, BQ-123, enrasentan updated in the last three years, counted by the country group of their listed sites. A trial with sites in several groups counts in each; 96 of 135 have all sites in one country.
Site counts are not patients per country, and trials registered only outside ClinicalTrials.gov are not covered. Where sites are is a fact about the record, not a judgement of its quality. Europe is the EU and EEA, the UK and Switzerland. 34 trials list no sites and are left out. Source: ClinicalTrials.gov, fetched live.
84 trials of drugs against EDNRA were stopped early; 10 give no reason
Terminated, withdrawn and suspended interventional trials of aprocitentan, clazosentan, sparsentan, macitentan, ambrisentan, bosentan, darusentan, zibotentan, avosentan, tezosentan, BQ-123, enrasentan, newest update first, live from ClinicalTrials.gov. Each reason is quoted exactly as the registry gives it; we do not sort reasons into safety, efficacy or business. Dates are the last registry update. Showing the 25 most recent of 84.
- terminated · 2026-09-25 · AstraZeneca · Part A: Placebo (matching zibotentan capsule & matching dapagliflozin tablet), Part A: zibotentan (dose B) + dapagliflozin, Part B: Placebo (matching zibotentan capsule & matching dapagliflozin tablet), Part B: placebo (matching zibotentan capsule) + dapagliflozin, Part B: zibotentan (dose A) + dapagliflozin, Part B: zibotentan (dose B) + dapagliflozin, Part B: zibotentan (dose C) + dapagliflozin“The study was ended earlier than planned due to a sponsor decision.”
- terminated · 2026-09-03 · Dr Sudarshan Rajagopal · Macitentan Tablets“Sponsor Requested”
- terminated · 2026-07-06 · United Therapeutics · matched by the registry's drug synonyms“Sponsor decision”
- suspended · 2026-06-15 · City of Hope Medical Center · Bosentan“Accrual Goal met”
- terminated · 2025-06-27 · Actelion · Macitentan“The Sponsor decided to stop the study for futility based on a recommendation by the IDMC following a pre-planned interim analysis”
- withdrawn · 2025-06-22 · Actelion · Macitentan“Due to Protocol AC-055B201 (MUSIC) not meeting it's primary end point”
- withdrawn · 2025-03-30 · Actelion · Macitentan“Sponsor Decision”
- withdrawn · 2025-02-20 · Franz Rischard, DO · matched by the registry's drug synonyms“Did not identify eligible candidates”
- terminated · 2025-02-04 · Actelion · Bosentan“Terminated due to slow enrollment”
- terminated · 2025-02-04 · Actelion · bosentan“Slow enrollment”
- terminated · 2025-02-04 · Actelion · macitentan 10 mg“the absence of a positive trend in all efficacy parameters indicates that macitentan 10 mg does not have a beneficial effect in patients with HFpEF and PVD.”
- terminated · 2025-02-04 · Actelion · Bosentan“Slow enrollment”
- terminated · 2025-02-04 · Actelion · Bosentan“To be compliant with the timelines as agreed with Paediatric Committee (PC) within the Paediatric Investigational Plan”
- terminated · 2025-02-04 · Actelion · Macitentan 3 mg, Macitentan 10 mg“company decision”
- terminated · 2025-02-03 · Actelion · matched by the registry's drug synonyms“The sponsor decided to terminate the study.”
- terminated · 2025-02-03 · Actelion · Bosentan“Slow enrollment”
- terminated · 2024-10-15 · Duke University · matched by the registry's drug synonyms“Project was completed”
- withdrawn · 2024-03-08 · University of Maryland, Baltimore · Macitentan 10mg“Study was cancelled by funding agency before starting enrollment.”
- terminated · 2024-03-05 · United Therapeutics · matched by the registry's drug synonyms“Sponsor Decision”
- withdrawn · 2024-02-20 · University of Missouri-Columbia · matched by the registry's drug synonyms“No participants enrolled”
- terminated · 2024-01-03 · United Therapeutics · matched by the registry's drug synonyms“Study closed due to limited availability of eligible subjects and competition by for enrollment by other studies”
- terminated · 2024-01-03 · United Therapeutics · matched by the registry's drug synonyms“Due to availability of eligible subjects at center and enrollment competition with other studies.”
- withdrawn · 2023-12-19 · United Therapeutics · matched by the registry's drug synonymsNo reason given
- terminated · 2023-10-31 · University of Mississippi Medical Center · Bosentan 125 mg“Despite significant effort, we were unable to obtain the supply of drugs and supplements needed for the various arms of this Clinical Trial.”
- terminated · 2023-03-21 · Noorik Biopharmaceuticals AG · Ambrisentan“Lack of recruitment”
Paediatric incentives and obligations for EDNRA's drugs
For aprocitentan, clazosentan, sparsentan, macitentan, ambrisentan, bosentan, darusentan, zibotentan, avosentan, tezosentan, BQ-123, enrasentan: what EMA and FDA publish about paediatric plans, orphan status and pediatric approvals, and how many registered studies take children. Each line is a yes, no or not published fact from the record linked under it; none of it is a forecast.
- FDA pediatric target list: no · EDNRA is not on the listRule: Target is on FDA's Pediatric Molecular Target List (relevant targets). Source: FDA Pediatric Molecular Target List (Listed as February 2026 on the FDA Pediatric Oncology page; the file served is 9.7.22_the_relevant_molecular_target_list.xlsx, whose Summary of Changes sheet is headed 'SEPTEMBER 2022 UPDATE' (footnote date 2022-09-01)), 2026-11-11
- EU paediatric investigation plan: yes · EMA/PE/0000226166 (sparsentan, Treatment of primary IgA nephropathy): PM: decision on the application for modification of an agreed PIP, 16 May 2025; EMEA-000434-PIP01-08-M10 (ambrisentan, Treatment of pulmonary arterial hypertension): PM: decision on the application for modification of an agreed PIP, 25 Oct 2024; EMEA-001032-PIP01-10-M07 (macitentan, Treatment of idiopathic pulmonary fibrosis;Treatment of pulmonary arterial hypertension;Treatment of systemic sclerosis): PM: decision on the application for modification of an agreed PIP, 1 Dec 2023; compliance positive 23 Feb 2024; EMEA-001984-PIP02-20-M01 (sparsentan, Treatment of focal segmental glomerulosclerosis): PM: decision on the application for modification of an agreed PIP, 21 Apr 2023; +8 moreRule: An EMA PIP decision (agreement, modification or waiver) lists the drug as its active substance, exact name. Source: EMA data download (dated snapshot), 2026-11-11
- EU orphan designation: yes · EU/3/09/707 withdrawn 28 Jan 2010: Treatment of idiopathic pulmonary fibrosis; EU/3/21/2428 withdrawn 20 May 2021: Treatment of functional single ventricle congenital heart disease; EU/3/21/2533 withdrawn 10 Dec 2021: Treatment of chronic thromboembolic pulmonary hypertension; EU/3/11/909 withdrawn 27 Sept 2011: Treatment of pulmonary arterial hypertension; +6 moreRule: An EU orphan designation lists the drug as its active substance (exact name) or belongs to a medicine that does; withdrawn and expired designations are listed with their status. Source: EMA data download (dated snapshot), 2026-11-11
- Authorised in the EU: yes · Ambrisentan Viatris (previously Ambrisentan Mylan): authorised since 20 Jun 2019; Macitentan Accord: authorised since 24 Sept 2025; Macitentan AccordPharma: authorised since 24 Sept 2025; Opsumit: authorised since 20 Dec 2013; +7 moreRule: An EMA medicine record lists the drug as its active substance or INN, exact name; national-only authorisations are not in this file. Source: EMA data download (dated snapshot), 2026-11-11
- FDA orphan designation: not published · FDA's orphan designation database turns away automated requests, so this app does not read it; search it by hand at the linkRule: Would be: an FDA orphan designation names the drug. Source: FDA orphan designations search
- FDA pediatric oncology approval: no · FDA's table lists no pediatric approval of aprocitentan, clazosentan, sparsentan, macitentan, ambrisentan, bosentan, darusentan, zibotentan, avosentan, tezosentan, bq-123, enrasentanRule: FDA's Pediatric Oncology Drug Approvals table names the drug. Source: FDA Pediatric Oncology Drug Approvals (content current as of 2026-03-03), 2026-11-12
- Registered studies open to children: yes · 59 interventional studies of aprocitentan, clazosentan, sparsentan, macitentan, ambrisentan, bosentan, darusentan, zibotentan, avosentan, tezosentan, bq-123, enrasentan include patients under 18; 4 are recruiting, not yet recruiting or activeRule: The registry's age group includes Child (birth to 17 years), which also counts studies that set no minimum age. Source: ClinicalTrials.gov search, 2026-11-12
Rare pediatric disease voucher. FDA, content current as of 29 Apr 2026: “As of February 3, 2026, enactment of the Consolidated Appropriations Act, 2026, the rare pediatric disease PRV program will sunset after September 30, 2029. FDA may not award any PRVs under this program after September 30, 2029.” Voucher sale prices are not shown here; they appear only as company-reported news. FDA: Rare Pediatric Disease Designation and Priority Review Voucher Programs
All 16 drugs and clinical candidates as a table
Each drug, stage and indication links to its Open Targets record, where the trials and approvals behind it are listed. Salt forms (for example a mesylate) are listed as Open Targets records them, as separate entries.