OncoSignal
Live from Open Targets Platform, fetched 2026-11-11 22:58 UTC. Every row links to its source record.

IDH2 isocitrate dehydrogenase (NADP(+)) 2

ENSG00000182054 · protein coding · Open Targets profile

Plays a role in intermediary metabolism and energy production (PubMed:19228619, PubMed:22416140). It may tightly associate or interact with the pyruvate dehydrogenase complex (PubMed:19228619, PubMed:22416140). {ECO:0000269|PubMed:19228619, ECO:0000269|PubMed:22416140}.

2 of the 4 drugs recorded against IDH2 are approved, and 1 more reached Phase 3

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.

2 small molecule drugs at Approved: VORASIDENIB, ENASIDENIB MESYLATE1 small molecule drug at Phase 3: ENASIDENIB1 small molecule drug at Stage not recorded: VORASIDENIB CITRATESmall molecule 4Approved 2Phase 3 1Stage not recorded 1

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 Withdrawn means the drug was pulled from the market or from development.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.

In 9 of 15 cancers, the evidence for IDH2 comes mainly from mutations found in tumours

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.

Inherited (germline) variantsMutations in tumours (somatic)
00.250.50.751gliomagliomaglioma: somatic 0.46glioma: germline 0.89acute myeloid leukemiaacute myeloid leukemiaacute myeloid leukemia: somatic 0.78acute myeloid leukemia: germline 0.32astrocytoma (excluding glioblastoma)astrocytoma (excluding glioblastoma)astrocytoma (excluding glioblastoma): somatic 0.46oligodendrogliomaoligodendrogliomamyeloid neoplasmmyeloid neoplasmmyeloid neoplasm: somatic 0.46myeloid neoplasm: germline 0.69myeloid leukemiamyeloid leukemiamyeloid leukemia: germline 0.73myelodysplastic syndromemyelodysplastic syndromemyelodysplastic syndrome: somatic 0.61Maffucci syndromeMaffucci syndromeMaffucci syndrome: somatic 0.44Ollier diseaseOllier diseaseOllier disease: germline 0.24Immunoblastic LymphadenopathyImmunoblastic LymphadenopathyImmunoblastic Lymphadenopathy: somatic 0.61olfactory neuroblastomaolfactory neuroblastomaolfactory neuroblastoma: somatic 0.61osteosarcomaosteosarcomaosteosarcoma: somatic 0.61sinonasal undifferentiated carcinomasinonasal undifferentiated carcinomasinonasal undifferentiated carcinoma: somatic 0.61acute myeloid leukemia by FAB classific…acute myeloid leukemia by FAB classificationanaplastic oligodendrogliomaanaplastic oligodendrogliomaanaplastic oligodendroglioma: somatic 0.61

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
PROTAC
Database Ubiquitination, Half-life Data, Small Molecule Binder

gnomAD loss of function constraint: o/e 0.35611000657081604 (upper bound 0.5280600190162659). 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 54 registry trials, updated in the last three years, of the most advanced drugs against IDH2 (vorasidenib, enasidenib), ranked by Phase 2+ trials. Live from ClinicalTrials.gov.

27 of 53 trials of drugs against IDH2 have sites only in United States

Registry trials of vorasidenib, enasidenib 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; 35 of 53 have all sites in one country.

Sites only in this groupSites here and elsewhere
United StatesUnited States: 27 trials with sites only hereUnited States: 13 trials with sites here and elsewhere40ChinaChina: 2 trials with sites only hereChina: 3 trials with sites here and elsewhere5EuropeEurope: 6 trials with sites only hereEurope: 9 trials with sites here and elsewhere15JapanJapan: 0 trials with sites only hereJapan: 2 trials with sites here and elsewhere2OtherOther: 2 trials with sites only hereOther: 14 trials with sites here and elsewhere16

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. 1 trials list no sites and are left out. Source: ClinicalTrials.gov, fetched live.

5 trials of drugs against IDH2 were stopped early

Terminated, withdrawn and suspended interventional trials of vorasidenib, enasidenib, 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 5 most recent of 5.

Paediatric incentives and obligations for IDH2's drugs

For vorasidenib, enasidenib: 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: yes · IDH2 is on the list. Membership alone neither requires nor waives a pediatric study.
  • EU paediatric investigation plan: yes · EMEA-001798-PIP02-16-M01 (Enasidenib, Treatment of acute myeloid leukaemia): PM: decision on the application for modification of an agreed PIP, 28 Oct 2022
    Rule: An EMA PIP decision (agreement, modification or waiver) lists the drug as its active substance, exact name. Source: EMA EMEA-001798-PIP02-16-M01, 2026-11-11
  • EU orphan designation: yes · EU/3/22/2737 positive 13 Jan 2023: Treatment of glioma; EU/3/16/1640 positive 28 Apr 2016: Treatment of acute myeloid leukaemia
    Rule: 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 · Voranigo: authorised since 17 Sept 2025, orphan medicine
    Rule: 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 EPAR Voranigo, 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 link
    Rule: Would be: an FDA orphan designation names the drug. Source: FDA orphan designations search
  • FDA pediatric oncology approval: yes · Vorasidenib [Voranigo], 6 Aug 2024: Treatment of adult and pediatric patients 12 years and older with Grade 2 astrocytoma or oligodendroglioma with a susceptible isocitrate dehydrogenase-1 (IDH1) or isocitrate dehydrogenase-2 (IDH2) mutation following surgery including biopsy, sub-total resection, or gross total resection
    Rule: FDA's Pediatric Oncology Drug Approvals table names the drug. Source: FDA Pediatric Oncology Drug Approvals (content current as of 2026-03-03), 2026-11-11
  • Registered studies open to children: yes · 9 interventional studies of vorasidenib, enasidenib include patients under 18; 9 are recruiting, not yet recruiting or active
    Rule: The registry's age group includes Child (birth to 17 years), which also counts studies that set no minimum age. Source: ClinicalTrials.gov search, 2026-11-11

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 4 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.

DrugTypeHighest stageIndicationsSource records
VORASIDENIBSmall moleculeapprovaloligodendroglioma, astrocytoma (excluding glioblastoma), glioma, acute myeloid leukemia by FAB classification, hematopoietic and lymphoid cell neoplasm, paraganglioma, low grade glioma, neoplasmOpen Targets · ChEMBL
ENASIDENIB MESYLATESmall moleculeapprovalacute myeloid leukemia by FAB classification, acute myeloid leukemiaOpen Targets · ChEMBL
ENASIDENIBSmall moleculephase 3acute myeloid leukemia, myelodysplastic syndrome, myelofibrosis, myeloproliferative disorder, leukemia, chondrosarcoma, olfactory neuroblastoma, sinonasal undifferentiated carcinoma, +9 moreOpen Targets · ChEMBL
VORASIDENIB CITRATESmall moleculeunknownneoplasmOpen Targets · ChEMBL