Gene

IDH1

isocitrate dehydrogenase (NADP(+)) 1

Explore in graph →CI-GENE-00005338HGNC:5382 ENSG00000138413 NCBI 3417 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (93)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 1–50 of 93 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
IDH1 Mutation7
(prognostic)	Chondrosarcoma	Prognostic	B	Supports Better Outcome	4	accepted	EID7725 In a retrospective study, the authors evaluated the association between IDH1/IDH2 mutation and prognosis in patients with high-grade central chondrosarcoma. Although no association was discovered betw…

Cohorts

Alteration frequency by cohort (105)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

cbioportalObserved105 cohort rows (showing 50, sorted by frequency) · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Low-Grade Gliomas (UCSF, Science 2014) lgg_ucsf_2014	Diffuse Glioma	Ssm	61	61

Literature

Linked publications (25)

25 per page, newest first.

pubmedPublishedShowing 1–25 of 25 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

Ivosidenib in IDH1-mutant, chemotherapy-refractory cholangiocarcinoma (ClarIDHy): a multicentre, randomised, double-blind, placebo-controlled, phase 3 study.
· Lancet Oncol · 2020 · PMID 32416072 · validated civic_curation pubmed
Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma.
· N Engl J Med · 2023 · PMID 37272516 · validated civic_curation pubmed
Durable Remissions with Ivosidenib in IDH1-Mutated Relapsed or Refractory AML.
· N Engl J Med · 2018 · PMID 29860938 · validated civic_curation pubmed
IDH1 and IDH2 mutations in gliomas.
· N Engl J Med · 2009 · PMID 19228619 · validated civic_curation pubmed

IDH1 R13229
(prognostic)	GlioblastomaCURATED_BROADER	Prognostic	B	Supports Better Outcome	5	accepted	EID480 In patients with a glioblastoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC	civic
〃	Anaplastic AstrocytomaALIAS	Prognostic	B	Supports Better Outcome	5	accepted	EID481 In patients with anaplastic astrocytoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC	civic
〃	Myelodysplastic SyndromeCURATED_BROADER	Prognostic	B	Supports Poor Outcome	4	accepted	EID374 IDH1 R132 mutation in patients suffering from myelodysplastic syndromes is associated with worse overall survival. PMID 20494930 · Thol et al., 2010 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Does Not Support N/A	3	accepted	EID160 Mutation status of IDH1 did not change event-free survival of patients with an NPM1 mutation. PMID 20805365 · Schnittger et al., 2010 · Open in CIViC	civic
〃	Anaplastic AstrocytomaALIAS	Prognostic	B	Supports Better Outcome	3	accepted	EID348 In patients with astrocytoma, the presence of IDH1 R132 mutation is prognostic for better survival compared to patients who harbor wild type mutation. PMID 19933982 · Dubbink et al., 2009 · Open in CIViC	civic
〃	GlioblastomaCURATED_BROADER	Prognostic	B	Supports Better Outcome	3	accepted	EID349 In patients with glioblastoma, those harboring IDH1 R132 mutation has higher overall survival compared to those who do not have IDH1 mutation. PMID 20127344 · Bleeker et al., 2010 · Open in CIViC	civic
〃	Glioma	Prognostic	B	Supports Better Outcome	3	submitted	EID4031 In a clinical study of 63 recurrent glioma patients, patients harboring IDH1 codon 132 mutation were associated with improved overall survival from initial diagnosis (56.5 vs. 21.3 months; P=0.001), a… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
(diagnostic)	Chondrosarcoma	Diagnostic	B	Supports Positive	3	submitted	EID11198 Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC) PMID 21598255 · Amary et al., 2011 · Open in CIViC	civic
(oncogenic)	Acute Myeloid LeukemiaCURATED_BROADER	Oncogenic	B	Supports Oncogenicity	3	submitted	EID12900 In this post hoc long-term follow-up of the phase 3 AGILE trial (NCT03173248; median follow-up 28.6 months; n=148), IDH1 R132 mutations served as the obligate driver alteration defining patient eligib… (full text at CIViC) PMID 40706052 · Montesinos et al., 2025 · Open in CIViC	civic
(diagnostic)	Acute Myeloid LeukemiaCURATED_BROADER	Diagnostic	B	Supports Positive	2	accepted	EID223 In leukemia patients, IDH1 R132 mutation is associated with adults and normal karyotype. PMID 20376086 · Ho et al., 2010 · Open in CIViC	civic
(prognostic)	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Does Not Support N/A	2	accepted	EID323 The complete remission and overall survival rates in patients with IDH1 R132 mutation is not significantly different from those who do not have this mutation. PMID 20368538 · Wagner et al., 2010 · Open in CIViC	civic
(diagnostic)	Chondrosarcoma	Diagnostic	B	Supports Positive	2	rejected	EID11197 Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC) PMID 21598255 · Amary et al., 2011 · Open in CIViC	civic
(predisposing)	Acute Myeloid LeukemiaCURATED_BROADER	Predisposing	C	Does Not Support Likely Pathogenic	3	submitted	EID7637 Out of 520 patient samples analyzed, 9.6% (n=50) had an IDH1 mutation. Of the patients with an IDH1 mutation, 44% (n=22) had a heterozygous R132H mutation, 42% (n=21) had a R132C mutation, 12% (n=6) h… (full text at CIViC) PMID 20625116 · Boissel et al., 2010 · Open in CIViC	civic
Bevacizumab	High-Grade Glioma, NOS	Predictive	B	Supports Sensitivity Response	2	submitted	EID2330 In a clinical study of 63 recurrent glioma patients, patients treated with bevacizumab monotherapy (n=11) and harboring IDH1 codon 132 mutation were associated with improved progression free survival … (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
Bevacizumab + SunitinibSubstitutes	High-Grade Glioma, NOS	Predictive	B	Supports Sensitivity Response	2	accepted	EID2020 In a clinical study of 63 recurrent glioma patients, the combined cohort of patients treated with either bevacizumab or sunitinib showed that those harboring IDH1 codon 132 mutation were associated wi… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
Cetuximab	GlioblastomaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	3	submitted	EID4032 In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
〃	GlioblastomaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	—	submitted	EID3718 In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
Dasatinib	Intrahepatic Cholangiocarcinoma	Predictive	D	Supports Sensitivity Response	4	accepted	EID6439 Through a high-throughput drug screen of a large panel of cancer cell lines, including 17 biliary tract cancers, this work found that IDH mutant (IDHm) ICC cells demonstrate a striking response to the… (full text at CIViC) PMID 27231123 · Saha et al., 2016 · Open in CIViC	civic
Ivosidenib	Cholangiocarcinoma	Predictive	A	Supports Sensitivity Response	5	accepted	EID10886 The multicenter randomized double-blind phase 3 study included patients with IDH1-mutant cholangiocarcinoma who had progressed on previous therapy. Patients were randomly assigned to Ivosidenib or pla… (full text at CIViC) PMID 32416072 · Abou-Alfa et al., 2020 · Open in CIViC	civic
〃	Chondrosarcoma	Predictive	A	Supports Sensitivity Response	2	submitted	EID11194 In a phase 1 study (NCT02073994), 21 patients with advanced IDH-mutant chondrosarcoma received the IDH1-mutant specific inhibitor ivosidenib. Plasma levels of the 2-HG oncometabolite decreased to nor… (full text at CIViC) PMID 32208957 · Tap et al., 2020 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	4	submitted	EID13077 In a multicenter phase I study, 33 patients with newly diagnosed IDH1 R132-mutated acute myeloid leukemia who were ineligible for standard therapy received ivosidenib 500 mg once daily. The CR+CRh rat… (full text at CIViC) PMID 31841594 · Roboz et al., 2020 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	3	submitted	EID13076 In a subgroup analysis of a multicenter phase I study, 17 patients with relapsed or refractory IDH1 R132-mutated acute myeloid leukemia who responded to ivosidenib 500 mg once daily subsequently under… (full text at CIViC) PMID 33772143 · DiNardo et al., 2021 · Open in CIViC	civic
〃	Myelodysplastic SyndromeCURATED_BROADER	Predictive	B	Supports Sensitivity Response	3	submitted	EID13287 In the relapsed or refractory myelodysplastic syndrome (MDS) substudy of the phase 1, open-label, single-arm, multinational first-in-human trial of ivosidenib (NCT02074839), adults with MDS carrying a… (full text at CIViC) PMID 38640348 · DiNardo et al., 2024 · Open in CIViC	civic
Azacitidine + IvosidenibCombination	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	A	Supports Sensitivity Response	5	submitted	EID12899 In the double-blind, placebo-controlled, phase III AGILE trial (NCT03173248), 146 patients with newly diagnosed IDH1-mutated acute myeloid leukemia (AML) who were ineligible for intensive induction ch… (full text at CIViC) PMID 35443108 · Montesinos et al., 2022 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	A	Supports Sensitivity Response	5	submitted	EID12901 This post hoc long-term follow-up analysis of the phase 3, double-blind, placebo-controlled AGILE trial (NCT03173248) reports updated outcomes for 148 patients with newly diagnosed IDH1-mutated AML in… (full text at CIViC) PMID 40706052 · Montesinos et al., 2025 · Open in CIViC	civic
Olutasidenib	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	4	submitted	EID13078 In a preplanned interim analysis of the pivotal phase 2 cohort of an open-label, multicenter phase 1/2 trial, 153 patients with relapsed or refractory IDH1 R132-mutated acute myeloid leukemia received… (full text at CIViC) PMID 36724515 · de Botton et al., 2023 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	3	submitted	EID13080 In a multicenter, open-label phase 1 study, 22 response-evaluable patients with relapsed or refractory IDH1-mutated acute myeloid leukemia received olutasidenib monotherapy. Nine patients achieved an … (full text at CIViC) PMID 36370742 · Watts et al., 2023 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	3	submitted	EID13091 In a phase 2 subgroup analysis of patients with IDH1 R132-mutated acute myeloid leukemia (AML) previously treated with venetoclax-based therapy, 16 patients received olutasidenib monotherapy at 150 mg… (full text at CIViC) PMID 38538632 · Cortes et al., 2024 · Open in CIViC	civic
Safusidenib	Glioma	Predictive	B	Supports Sensitivity Response	3	submitted	EID7448 In a phase1 study, patients with Glioma harboring IDH1-R132X mutation were treated with selective IDH1-R132X inhibitor DS-1001b. The response rate was 17.1% (6/35) in patients with enhancing lesion a… (full text at CIViC) Open in CIViC	civic

IDH1 R13229

(prognostic)

GlioblastomaCURATED_BROADER

Prognostic

Supports Better Outcome

accepted

EID480

In patients with a glioblastoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients.

PMID 19228619 · Yan et al., 2009 · Open in CIViC

civic

〃

Anaplastic AstrocytomaALIAS

Prognostic

Supports Better Outcome

accepted

EID481

In patients with anaplastic astrocytoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients.

PMID 19228619 · Yan et al., 2009 · Open in CIViC

civic

〃

Myelodysplastic SyndromeCURATED_BROADER

Prognostic

Supports Poor Outcome

accepted

EID374

IDH1 R132 mutation in patients suffering from myelodysplastic syndromes is associated with worse overall survival.

PMID 20494930 · Thol et al., 2010 · Open in CIViC

civic

〃

Acute Myeloid LeukemiaCURATED_BROADER

Prognostic

Does Not Support N/A

accepted

EID160

Mutation status of IDH1 did not change event-free survival of patients with an NPM1 mutation.

PMID 20805365 · Schnittger et al., 2010 · Open in CIViC

civic

〃

Anaplastic AstrocytomaALIAS

Prognostic

Supports Better Outcome

accepted

EID348

In patients with astrocytoma, the presence of IDH1 R132 mutation is prognostic for better survival compared to patients who harbor wild type mutation.

PMID 19933982 · Dubbink et al., 2009 · Open in CIViC

civic

〃

GlioblastomaCURATED_BROADER

Prognostic

Supports Better Outcome

accepted

EID349

In patients with glioblastoma, those harboring IDH1 R132 mutation has higher overall survival compared to those who do not have IDH1 mutation.

PMID 20127344 · Bleeker et al., 2010 · Open in CIViC

civic

〃

Glioma

Prognostic

Supports Better Outcome

submitted

EID4031

In a clinical study of 63 recurrent glioma patients, patients harboring IDH1 codon 132 mutation were associated with improved overall survival from initial diagnosis (56.5 vs. 21.3 months; P=0.001), a… (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

(diagnostic)

Chondrosarcoma

Diagnostic

Supports Positive

submitted

EID11198

Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC)

PMID 21598255 · Amary et al., 2011 · Open in CIViC

civic

(oncogenic)

Acute Myeloid LeukemiaCURATED_BROADER

Oncogenic

Supports Oncogenicity

submitted

EID12900

In this post hoc long-term follow-up of the phase 3 AGILE trial (NCT03173248; median follow-up 28.6 months; n=148), IDH1 R132 mutations served as the obligate driver alteration defining patient eligib… (full text at CIViC)

PMID 40706052 · Montesinos et al., 2025 · Open in CIViC

civic

(diagnostic)

Acute Myeloid LeukemiaCURATED_BROADER

Diagnostic

Supports Positive

accepted

EID223

In leukemia patients, IDH1 R132 mutation is associated with adults and normal karyotype.

PMID 20376086 · Ho et al., 2010 · Open in CIViC

civic

(prognostic)

Acute Myeloid LeukemiaCURATED_BROADER

Prognostic

Does Not Support N/A

accepted

EID323

The complete remission and overall survival rates in patients with IDH1 R132 mutation is not significantly different from those who do not have this mutation.

PMID 20368538 · Wagner et al., 2010 · Open in CIViC

civic

(diagnostic)

Chondrosarcoma

Diagnostic

Supports Positive

rejected

EID11197

PMID 21598255 · Amary et al., 2011 · Open in CIViC

civic

(predisposing)

Acute Myeloid LeukemiaCURATED_BROADER

Predisposing

Does Not Support Likely Pathogenic

submitted

EID7637

Out of 520 patient samples analyzed, 9.6% (n=50) had an IDH1 mutation. Of the patients with an IDH1 mutation, 44% (n=22) had a heterozygous R132H mutation, 42% (n=21) had a R132C mutation, 12% (n=6) h… (full text at CIViC)

PMID 20625116 · Boissel et al., 2010 · Open in CIViC

civic

Bevacizumab

High-Grade Glioma, NOS

Predictive

Supports Sensitivity Response

submitted

EID2330

In a clinical study of 63 recurrent glioma patients, patients treated with bevacizumab monotherapy (n=11) and harboring IDH1 codon 132 mutation were associated with improved progression free survival … (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

Bevacizumab + SunitinibSubstitutes

High-Grade Glioma, NOS

Predictive

Supports Sensitivity Response

accepted

EID2020

In a clinical study of 63 recurrent glioma patients, the combined cohort of patients treated with either bevacizumab or sunitinib showed that those harboring IDH1 codon 132 mutation were associated wi… (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

Cetuximab

GlioblastomaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID4032

In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

〃

GlioblastomaCURATED_BROADER

Predictive

Supports Sensitivity Response

—

submitted

EID3718

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

Dasatinib

Intrahepatic Cholangiocarcinoma

Predictive

Supports Sensitivity Response

accepted

EID6439

Through a high-throughput drug screen of a large panel of cancer cell lines, including 17 biliary tract cancers, this work found that IDH mutant (IDHm) ICC cells demonstrate a striking response to the… (full text at CIViC)

PMID 27231123 · Saha et al., 2016 · Open in CIViC

civic

Ivosidenib

Cholangiocarcinoma

Predictive

Supports Sensitivity Response

accepted

EID10886

The multicenter randomized double-blind phase 3 study included patients with IDH1-mutant cholangiocarcinoma who had progressed on previous therapy. Patients were randomly assigned to Ivosidenib or pla… (full text at CIViC)

PMID 32416072 · Abou-Alfa et al., 2020 · Open in CIViC

civic

〃

Chondrosarcoma

Predictive

Supports Sensitivity Response

submitted

EID11194

In a phase 1 study (NCT02073994), 21 patients with advanced IDH-mutant chondrosarcoma received the IDH1-mutant specific inhibitor ivosidenib. Plasma levels of the 2-HG oncometabolite decreased to nor… (full text at CIViC)

PMID 32208957 · Tap et al., 2020 · Open in CIViC

civic

〃

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID13077

In a multicenter phase I study, 33 patients with newly diagnosed IDH1 R132-mutated acute myeloid leukemia who were ineligible for standard therapy received ivosidenib 500 mg once daily. The CR+CRh rat… (full text at CIViC)

PMID 31841594 · Roboz et al., 2020 · Open in CIViC

civic

〃

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID13076

In a subgroup analysis of a multicenter phase I study, 17 patients with relapsed or refractory IDH1 R132-mutated acute myeloid leukemia who responded to ivosidenib 500 mg once daily subsequently under… (full text at CIViC)

PMID 33772143 · DiNardo et al., 2021 · Open in CIViC

civic

〃

Myelodysplastic SyndromeCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID13287

In the relapsed or refractory myelodysplastic syndrome (MDS) substudy of the phase 1, open-label, single-arm, multinational first-in-human trial of ivosidenib (NCT02074839), adults with MDS carrying a… (full text at CIViC)

PMID 38640348 · DiNardo et al., 2024 · Open in CIViC

civic

Azacitidine + IvosidenibCombination

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID12899

In the double-blind, placebo-controlled, phase III AGILE trial (NCT03173248), 146 patients with newly diagnosed IDH1-mutated acute myeloid leukemia (AML) who were ineligible for intensive induction ch… (full text at CIViC)

PMID 35443108 · Montesinos et al., 2022 · Open in CIViC

civic

〃

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID12901

This post hoc long-term follow-up analysis of the phase 3, double-blind, placebo-controlled AGILE trial (NCT03173248) reports updated outcomes for 148 patients with newly diagnosed IDH1-mutated AML in… (full text at CIViC)

PMID 40706052 · Montesinos et al., 2025 · Open in CIViC

civic

Olutasidenib

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID13078

In a preplanned interim analysis of the pivotal phase 2 cohort of an open-label, multicenter phase 1/2 trial, 153 patients with relapsed or refractory IDH1 R132-mutated acute myeloid leukemia received… (full text at CIViC)

PMID 36724515 · de Botton et al., 2023 · Open in CIViC

civic

〃

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID13080

In a multicenter, open-label phase 1 study, 22 response-evaluable patients with relapsed or refractory IDH1-mutated acute myeloid leukemia received olutasidenib monotherapy. Nine patients achieved an … (full text at CIViC)

PMID 36370742 · Watts et al., 2023 · Open in CIViC

civic

〃

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID13091

In a phase 2 subgroup analysis of patients with IDH1 R132-mutated acute myeloid leukemia (AML) previously treated with venetoclax-based therapy, 16 patients received olutasidenib monotherapy at 150 mg… (full text at CIViC)

PMID 38538632 · Cortes et al., 2024 · Open in CIViC

civic

Safusidenib

Glioma

Predictive

Supports Sensitivity Response

submitted

EID7448

In a phase1 study, patients with Glioma harboring IDH1-R132X mutation were treated with selective IDH1-R132X inhibitor DS-1001b. The response rate was 17.1% (6/35) in patients with enhancing lesion a… (full text at CIViC)

Open in CIViC

civic

IDH1 R132 + IDH1 R132L1
(oncogenic)	Acute Myeloid LeukemiaCURATED_BROADER	Oncogenic	B	Supports Oncogenicity	3	submitted	EID12898 In the phase 3 AGILE trial enrolling patients with newly diagnosed IDH1-mutated AML who were ineligible for intensive induction chemotherapy, IDH1 R132 mutations (confirmed by FDA-approved Abbott Real… (full text at CIViC) PMID 35443108 · Montesinos et al., 2022 · Open in CIViC	civic

IDH1 R132 + IDH1 R132L1

(oncogenic)

Acute Myeloid LeukemiaCURATED_BROADER

Oncogenic

Supports Oncogenicity

submitted

EID12898

In the phase 3 AGILE trial enrolling patients with newly diagnosed IDH1-mutated AML who were ineligible for intensive induction chemotherapy, IDH1 R132 mutations (confirmed by FDA-approved Abbott Real… (full text at CIViC)

PMID 35443108 · Montesinos et al., 2022 · Open in CIViC

civic

IDH1 R132C10
(diagnostic)	Acute Myeloid LeukemiaCURATED_BROADER	Diagnostic	B	Supports Positive	3	accepted	EID224 IDH1 R132 mutation is associated with patients of older age, high platelet count during diagnosis, cytogenic normalcy and NPM1 mutation. PMID 20538800 · Abbas et al., 2010 · Open in CIViC	civic
(prognostic)	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Does Not Support N/A	2	accepted	EID324 IDH1 R132 mutation in patients with AML is not associated with any prognostic value compared to patients with wild-type IDH1. PMID 20538800 · Abbas et al., 2010 · Open in CIViC	civic
AGI-5198	High-Grade Glioma, NOS	Predictive	D	Supports Sensitivity Response	2	accepted	EID2329 In an experimental in vitro study, glioma cells expressing IDH1 R132C were associated with sensitivity to AGI-5198 (IC50: 0.16M vs. IC50 > 100.00M), as compared to cells expressing wild-type IDH1. Sen… (full text at CIViC) PMID 23558169 · Rohle et al., 2013 · Open in CIViC	civic
BPTES	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	3	accepted	EID2332 In an experimental study of acute myeloid leukemia (AML) cell lines expressing IDH1 R132C, IDH1 R132G, or IDH1 R132H mutations were associated with increased sensitivity to small molecule glutaminase … (full text at CIViC) PMID 24333121 · Emadi et al., 2014 · Open in CIViC	civic
GSK321	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	3	accepted	EID732 Newly developed allosteric inhibitors (GSK321) of IDH1 led to granulocytic differentiation in-vitro and in-vivo. PMID 26436839 · Okoye-Okafor et al., 2015 · Open in CIViC	civic
Ivosidenib	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	B	Supports Sensitivity Response	2	accepted	EID2331 In a clinical study of 17 relapsed or refractory AML patients harboring IDH1 mutations, these patients were associated with response to AG-120 monotherapy; 4/7 patients who achieved a response experie… (full text at CIViC) PMID 25583779 · 2015 · Open in CIViC	civic
Olaparib	Malignant NeoplasmALIAS	Predictive	D	Supports Sensitivity Response	3	accepted	EID10144 IDH1 R132C mutation confers a switch of function mutation where the mutated protein produces 2-hydroxyglutarate (2HG), rather than α-ketoglutarate (αKG). The novel metabolite induces a homologous reco… (full text at CIViC) PMID 28148839 · Sulkowski et al., 2017 · Open in CIViC	civic
Temozolomide	Brain GliomaUNRESOLVED	Predictive	B	Supports Sensitivity Response	2	accepted	EID2327 In a study of 189 low-grade gliomas, IDH (IDH1 or IDH2) mutations were found in 132 patients (70%). IDH mutations were associated with improved rate of response to temozolomide as compared to patients… (full text at CIViC) PMID 20975057 · Houillier et al., 2010 · Open in CIViC	civic
〃	Brain GliomaUNRESOLVED	Predictive	B	Supports Sensitivity Response	—	rejected	EID2326 IDH1 mutations at R132H are the most frequent mutations accounting for 70- 90% of all IDH1 mutations in secondary glioblastoma and 5% of primary glioblastomas. PMID 18772396 · Parsons et al., 2008 · Open in CIViC	civic
〃	Brain GliomaUNRESOLVED	Predictive	B	Supports Sensitivity Response	—	rejected	EID2328 Isocitrate dehydrogenases (IDH) are enzymes of the citric acid cycle catalyzing the oxidative decarboxylation of isocitrate to 2-oxoglutarate. IDH1 mutations at R132H are the most frequent mutations a… (full text at CIViC) PMID 21625441 · Krell et al., 2011 · Open in CIViC	civic

IDH1 R132C10

(diagnostic)

Acute Myeloid LeukemiaCURATED_BROADER

Diagnostic

Supports Positive

accepted

EID224

IDH1 R132 mutation is associated with patients of older age, high platelet count during diagnosis, cytogenic normalcy and NPM1 mutation.

PMID 20538800 · Abbas et al., 2010 · Open in CIViC

civic

(prognostic)

Acute Myeloid LeukemiaCURATED_BROADER

Prognostic

Does Not Support N/A

accepted

EID324

IDH1 R132 mutation in patients with AML is not associated with any prognostic value compared to patients with wild-type IDH1.

PMID 20538800 · Abbas et al., 2010 · Open in CIViC

civic

AGI-5198

High-Grade Glioma, NOS

Predictive

Supports Sensitivity Response

accepted

EID2329

In an experimental in vitro study, glioma cells expressing IDH1 R132C were associated with sensitivity to AGI-5198 (IC50: 0.16M vs. IC50 > 100.00M), as compared to cells expressing wild-type IDH1. Sen… (full text at CIViC)

PMID 23558169 · Rohle et al., 2013 · Open in CIViC

civic

BPTES

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

accepted

EID2332

In an experimental study of acute myeloid leukemia (AML) cell lines expressing IDH1 R132C, IDH1 R132G, or IDH1 R132H mutations were associated with increased sensitivity to small molecule glutaminase … (full text at CIViC)

PMID 24333121 · Emadi et al., 2014 · Open in CIViC

civic

GSK321

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

accepted

EID732

Newly developed allosteric inhibitors (GSK321) of IDH1 led to granulocytic differentiation in-vitro and in-vivo.

PMID 26436839 · Okoye-Okafor et al., 2015 · Open in CIViC

civic

Ivosidenib

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

accepted

EID2331

In a clinical study of 17 relapsed or refractory AML patients harboring IDH1 mutations, these patients were associated with response to AG-120 monotherapy; 4/7 patients who achieved a response experie… (full text at CIViC)

PMID 25583779 · 2015 · Open in CIViC

civic

Olaparib

Malignant NeoplasmALIAS

Predictive

Supports Sensitivity Response

accepted

EID10144

IDH1 R132C mutation confers a switch of function mutation where the mutated protein produces 2-hydroxyglutarate (2HG), rather than α-ketoglutarate (αKG). The novel metabolite induces a homologous reco… (full text at CIViC)

PMID 28148839 · Sulkowski et al., 2017 · Open in CIViC

civic

Temozolomide

Brain GliomaUNRESOLVED

Predictive

Supports Sensitivity Response

accepted

EID2327

In a study of 189 low-grade gliomas, IDH (IDH1 or IDH2) mutations were found in 132 patients (70%). IDH mutations were associated with improved rate of response to temozolomide as compared to patients… (full text at CIViC)

PMID 20975057 · Houillier et al., 2010 · Open in CIViC

civic

〃

Brain GliomaUNRESOLVED

Predictive

Supports Sensitivity Response

—

rejected

EID2326

IDH1 mutations at R132H are the most frequent mutations accounting for 70- 90% of all IDH1 mutations in secondary glioblastoma and 5% of primary glioblastomas.

PMID 18772396 · Parsons et al., 2008 · Open in CIViC

civic

〃

Brain GliomaUNRESOLVED

Predictive

Supports Sensitivity Response

—

rejected

EID2328

Isocitrate dehydrogenases (IDH) are enzymes of the citric acid cycle catalyzing the oxidative decarboxylation of isocitrate to 2-oxoglutarate. IDH1 mutations at R132H are the most frequent mutations a… (full text at CIViC)

PMID 21625441 · Krell et al., 2011 · Open in CIViC

civic

IDH1 R132C + IDH1 R132H2
(oncogenic)	Acute Myeloid LeukemiaCURATED_BROADER	Oncogenic	B	Supports Oncogenicity	3	submitted	EID12902 Popovici-Muller et al. report that IDH1 R132 mutations are gain-of-function alterations that result in production of the oncometabolite D-2-hydroxyglutarate (2-HG). The authors state that elevated 2-H… (full text at CIViC) PMID 29670690 · Popovici-Muller et al., 2018 · Open in CIViC	civic
Ivosidenib	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	4	accepted	EID12903 Popovici-Muller et al. reported preliminary efficacy data from an ongoing phase 1 study of ivosidenib (AG-120) monotherapy in patients with IDH1-mutated malignancies. In patients with relapsed or refr… (full text at CIViC) PMID 29670690 · Popovici-Muller et al., 2018 · Open in CIViC	civic

IDH1 R132C + IDH1 R132H2

(oncogenic)

Acute Myeloid LeukemiaCURATED_BROADER

Oncogenic

Supports Oncogenicity

submitted

EID12902

Popovici-Muller et al. report that IDH1 R132 mutations are gain-of-function alterations that result in production of the oncometabolite D-2-hydroxyglutarate (2-HG). The authors state that elevated 2-H… (full text at CIViC)

PMID 29670690 · Popovici-Muller et al., 2018 · Open in CIViC

civic

Ivosidenib

Acute Myeloid LeukemiaCURATED_BROADER

Predictive

Supports Sensitivity Response

accepted

EID12903

Popovici-Muller et al. reported preliminary efficacy data from an ongoing phase 1 study of ivosidenib (AG-120) monotherapy in patients with IDH1-mutated malignancies. In patients with relapsed or refr… (full text at CIViC)

PMID 29670690 · Popovici-Muller et al., 2018 · Open in CIViC

civic

IDH1 R132C + IDH1 R132G + IDH1 R132H + IDH1 R132L + IDH1 R132S1
Vorasidenib	Low Grade Glioma	Predictive	A	Supports Sensitivity Response	5	accepted	EID11508 This phase 3 clinical trial was conducted to assess the progression free survival in patients with IDH-mutant gliomas treated with Vorasidenib, an oral brain-penetrant inhibitor, which showed prelimin… (full text at CIViC) PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC	civic

IDH1 R132C + IDH1 R132G + IDH1 R132H + IDH1 R132L + IDH1 R132S1

Vorasidenib

Low Grade Glioma

Predictive

Supports Sensitivity Response

accepted

EID11508

This phase 3 clinical trial was conducted to assess the progression free survival in patients with IDH-mutant gliomas treated with Vorasidenib, an oral brain-penetrant inhibitor, which showed prelimin… (full text at CIViC)

PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC

civic