{"entity":{"id":"idh1-r132","kind":"biomarker","name":"IDH1 R132 mutation","aka":["IDH1 R132H","IDH1 R132C","IDH1 mutation","IDH1-mutant","IDH1 R132","mIDH1","susceptible IDH1 mutation"],"tldr":"IDH1 R132 mutations turn a metabolic enzyme into a producer of the oncometabolite 2-HG. They define lower-grade gliomas and occur in acute myeloid leukaemia and bile duct cancer, each with an approved IDH1 inhibitor.","summary":"Codon 132 substitutions (R132H in over 90 percent of gliomas; R132C, G, S and L more often in AML and cholangiocarcinoma) are detected by IHC for R132H in glioma and by PCR or sequencing elsewhere. Ivosidenib (Tibsovo) is labelled for AML (newly diagnosed unfit, and relapsed or refractory), MDS and previously treated cholangiocarcinoma with a susceptible IDH1 mutation as detected by an FDA-approved test; olutasidenib (Rezlidhia) for relapsed or refractory AML with a susceptible IDH1 mutation; vorasidenib (Voranigo, 2024) for grade 2 astrocytoma or oligodendroglioma with a susceptible IDH1 or IDH2 mutation after surgery. The Abbott RealTime IDH1 and Oncomine Dx Target Test are the listed companion diagnostics.","asOf":"2026-09-23","links":[{"label":"TIBSOVO prescribing information (DailyMed)","url":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=65d254c0-67ad-42c4-b972-ad463b755b2d"},{"label":"VORANIGO prescribing information (DailyMed)","url":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=31405fee-55b7-4857-987e-2724ee76be84"}],"tags":["biomarker","idh"],"related":["idh2-mutation","1p19q-codeletion-readout","mgmt-promoter-methylation"],"cancers":["aml","aml-idh","cholangiocarcinoma","glioblastoma","idh-mutant-astrocytoma","oligodendroglioma","mds"],"sections":[],"technologies":[],"targets":[],"drugs":["ivosidenib","olutasidenib","vorasidenib","oncomine-dx-target-test"],"companies":[],"institutions":[],"pathways":[],"terms":["ngs"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"target":"idh","measurement":"sequencing-variant","scoringRule":{"text":"A susceptible IDH1 codon 132 substitution (R132C, R132H, R132G, R132S, R132L) by PCR or sequencing of blood, marrow or tumour tissue; R132H can be screened by immunohistochemistry in glioma.","quote":"R132 mutations (R132C, R132H, R132G, R132S, and R132L)","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools","sourceLabel":"FDA: List of FDA-Authorized Companion Diagnostic Devices"},"thresholds":[{"value":"Susceptible IDH1 mutation","drugId":"ivosidenib","cancerId":"aml-idh","regulator":"FDA","source":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=65d254c0-67ad-42c4-b972-ad463b755b2d","quote":"TIBSOVO is an isocitrate dehydrogenase-1 (IDH1) inhibitor indicated for patients with a susceptible IDH1 mutation as detected by an FDA-approved test with: Newly Diagnosed Acute Myeloid Leukemia (AML) In combination with azacitidine or as monotherapy for the treatment of newly diagnosed AML in adults 75 years or older, or who have comorbidities that preclude use of intensive induction","status":"current"},{"value":"Susceptible IDH1 mutation","drugId":"olutasidenib","cancerId":"aml-idh","regulator":"FDA","source":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=4a0c7c8b-b95f-455d-9600-b7351e4397fe","quote":"REZLIDHIA is indicated for the treatment of adult patients with relapsed or refractory acute myeloid leukemia (AML) with a susceptible isocitrate dehydrogenase-1 (IDH1) mutation as detected by an FDA-approved test","status":"current"},{"value":"Susceptible IDH1 or IDH2 mutation, grade 2 glioma after surgery","drugId":"vorasidenib","cancerId":"idh-mutant-astrocytoma","regulator":"FDA","source":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=31405fee-55b7-4857-987e-2724ee76be84","quote":"VORANIGO is an isocitrate dehydrogenase-1 (IDH1) and isocitrate dehydrogenase-2 (IDH2) inhibitor indicated for the treatment of adult and pediatric patients 12 years and older with Grade 2 astrocytoma or oligodendroglioma with a susceptible IDH1 or IDH2 mutation, as detected by an FDA-approved test, following surgery including biopsy, sub-total resection, or gross total resection.","status":"current"}],"tests":["foundationone-cdx","foundationone-heme","tempus-xt","trusight-oncology-comprehensive","neotype-profiles"],"assays":["idh1-abbott","oncomine-dx"],"companionDiagnostics":[{"device":"Abbott RealTime IDH1","maker":"Abbott Molecular","companyId":"abbott","drugs":["ivosidenib"],"indication":"Acute Myeloid Leukemia - Peripheral Blood or Bone Marrow","pma":"P170041 (07/20/2018)","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools"},{"device":"Abbott RealTime IDH1","maker":"Abbott Molecular","companyId":"abbott","drugs":["olutasidenib"],"indication":"Acute Myeloid Leukemia - Peripheral Blood or Bone Marrow","pma":"P170041/S006 (12/01/2022)","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools"},{"device":"Oncomine Dx Target Test","maker":"Life Technologies (Thermo Fisher Scientific)","companyId":"thermo-fisher","drugs":["ivosidenib"],"indication":"Cholangiocarcinoma - Tissue","pma":"P160045/S028 (08/25/2021)","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools"},{"device":"Oncomine Dx Target Test","maker":"Life Technologies (Thermo Fisher Scientific)","companyId":"thermo-fisher","drugs":["vorasidenib"],"indication":"Astrocytoma and Oligodendroglioma - Tissue","pma":"P160045/S046 (09/18/2024)","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools"}],"forPatient":"An IDH1 mutation opens a targeted tablet in three settings: ivosidenib or olutasidenib in acute myeloid leukaemia, ivosidenib in bile duct cancer after chemotherapy, and vorasidenib in a grade 2 glioma after surgery, where it can delay the need for radiotherapy and chemotherapy. In glioma the mutation also carries a better outlook than the same tumour without it."},"route":"/biomarkers/idh1-r132/","neighbours":{"biomarker":[{"id":"1p19q-codeletion-readout","kind":"biomarker","name":"1p/19q codeletion","route":"/biomarkers/1p19q-codeletion-readout/"},{"id":"h3-k27m","kind":"biomarker","name":"H3 K27M mutation","route":"/biomarkers/h3-k27m/"},{"id":"idh2-mutation","kind":"biomarker","name":"IDH2 mutation (R140 and R172)","route":"/biomarkers/idh2-mutation/"},{"id":"mgmt-promoter-methylation","kind":"biomarker","name":"MGMT promoter methylation","route":"/biomarkers/mgmt-promoter-methylation/"},{"id":"npm1-mutation","kind":"biomarker","name":"NPM1 mutation","route":"/biomarkers/npm1-mutation/"},{"id":"tp53-del17p","kind":"biomarker","name":"TP53 mutation and del(17p)","route":"/biomarkers/tp53-del17p/"}],"cancer":[{"id":"aml","kind":"cancer","name":"Acute myeloid leukaemia","route":"/cancers/aml/"},{"id":"idh-mutant-astrocytoma","kind":"cancer","name":"Astrocytoma, IDH-mutant (grades 2 to 4)","route":"/cancers/idh-mutant-astrocytoma/"},{"id":"cholangiocarcinoma","kind":"cancer","name":"Biliary tract cancer (cholangiocarcinoma)","route":"/cancers/cholangiocarcinoma/"},{"id":"glioblastoma","kind":"cancer","name":"Glioma & glioblastoma","route":"/cancers/glioblastoma/"},{"id":"aml-idh","kind":"cancer","name":"IDH1- and IDH2-mutated acute myeloid leukaemia","route":"/cancers/aml-idh/"},{"id":"mds","kind":"cancer","name":"Myelodysplastic syndromes / neoplasms (MDS)","route":"/cancers/mds/"},{"id":"oligodendroglioma","kind":"cancer","name":"Oligodendroglioma, IDH-mutant and 1p/19q-codeleted","route":"/cancers/oligodendroglioma/"}],"drug":[{"id":"ivosidenib","kind":"drug","name":"Ivosidenib","route":"/drugs/ivosidenib/"},{"id":"olutasidenib","kind":"drug","name":"Olutasidenib","route":"/drugs/olutasidenib/"},{"id":"oncomine-dx-target-test","kind":"drug","name":"Oncomine Dx Target Test","route":"/drugs/oncomine-dx-target-test/"},{"id":"vorasidenib","kind":"drug","name":"Vorasidenib","route":"/drugs/vorasidenib/"}],"term":[{"id":"ngs","kind":"term","name":"Next-generation sequencing (NGS)","route":"/terms/ngs/"}],"target":[{"id":"idh","kind":"target","name":"IDH1 / IDH2","route":"/targets/idh/"}]}}