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Profile Name	IDH1 R132G
Gene Variant Detail	IDH1 R132G (gain of function)
Relevant Treatment Approaches	IDH Inhibitor (Pan) IDH1 Inhibitor

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Showing 1 to 11 of 11 entries

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Molecular Profile	Indication/Tumor Type	Response Type	Relevant Treatment Approaches	Therapy Name	Approval Status	Evidence Type	Efficacy Evidence	References
IDH1 R132G	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) treatment resulted in complete remission (CR) in 21.6% (27/125), CR with partial hematological recovery (CRh) in 8.8% (11/125), and overall response (OR) in 41.6% (52/125) of patients with relapsed or refractory acute myeloid leukemia harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test (PMID: 29860938; NCT02074839).	detail... 29860938 detail...
IDH1 R132G	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) treatment resulted in complete remission (CR) in 28.6% (8/28), CR with partial hematological recovery (CRh) in 14.3% (4/28) of patients age 75 or older with untreated acute myeloid leukemia harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test, with a median treatment duration of 4.3 months (PMID: 29860938; NCT02074839).	detail... 29860938 detail...
IDH1 R132G	cholangiocarcinoma	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase III (ClarIDHy) trial that supported FDA approval, Tibsovo (ivosidenib) treatment significantly improved median progression-free survival (2.7 vs 1.4 mo, HR=0.37, p<0.001) and prolonged median overall survival (10.8 vs 9.7 mo, HR=0.69, p=0.06) compared to placebo in patients with advanced cholangiocarcinoma harboring IDH1 mutations including R132C/H/L/G/S, resulted in favorable objective response rate (2%, 3/124 vs 0%, 0/61) and stable disease rate (51% vs 28%) (PMID: 32416072; NCT02989857).	detail... 32416072 detail...
IDH1 R132G	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Azacitidine + Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase III trial (AGILE) that supported FDA approval, Tibsovo (ivosidenib) and Vidaza (azacitidine) combination therapy significantly improved event-free survival (HR 0.33, p=0.002) and median overall survival (24.0 vs 7.9 mo, HR 0.44, p=0.001) compared to Vidaza (azacitidine) plus placebo in patients with newly diagnosed acute myeloid leukemia harboring IDH1 mutations including R132C/H/G/L/S (PMID: 35443108; NCT03173248).	35443108 detail... detail...
IDH1 R132G	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Olutasidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase II trial (Study 2102-HEM-101) that supported FDA approval, Rezlidhia (olutasidenib) treatment resulted in an objective response rate of 46% (57/123, 37 complete remission (CR), 4 CR with partial hematologic recovery, 14 CR with incomplete recovery, 1 morphologic leukemia-free state, 1 partial response) in patients with relapsed/refractory acute myeloid leukemia harboring a susceptible IDH1 mutation (R132G/C/H/L/S) (J Clin Oncol 39, no. 15_suppl (May 20, 2021) 7006; NCT02719574).	detail... detail... detail...
IDH1 R132G	myelodysplastic syndrome	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) was tolerated and resulted in a complete response (CR) in 44% (7/16), partial response (PR) in 6% (1/16), and marrow CR in 31% (5/16) of patients with relapsed or refractory myelodysplastic syndrome harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test, with a hematologic improvement in >/=1 lineages achieved by 69% (11/16) of patients (J Clin Oncol 40, no. 16_suppl (June 01, 2022) 7053; NCT02074839).	detail... detail... detail...
IDH1 R132G	oligodendroglioma	sensitive	IDH Inhibitor (Pan)	Vorasidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase III trial l (INDIGO) that supported FDA approval, Voranigo (vorasidenib) treatment significantly improved progression-free survival (27.7 vs 11.1 months, HR 0.39, p<0.001) and time to next intervention (HR 0.26, p<0.001) compared to placebo in adult and pediatric patients 12 years and older with WHO grade 2 oligodendroglioma or astrocytoma harboring susceptible IDH1 or IDH2 mutations, including IDH1 R132H/C/G/L/S (PMID: 37272516; NCT04164901).	37272516 detail... detail...
IDH1 R132G	astrocytoma, IDH-mutant, grade 2	sensitive	IDH Inhibitor (Pan)	Vorasidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase III trial l (INDIGO) that supported FDA approval, Voranigo (vorasidenib) treatment significantly improved progression-free survival (27.7 vs 11.1 months, HR 0.39, p<0.001) and time to next intervention (HR 0.26, p<0.001) compared to placebo in adult and pediatric patients 12 years and older with WHO grade 2 astrocytoma or oligodendroglioma harboring susceptible IDH1 or IDH2 mutations, including IDH1 R132H/C/G/L/S (PMID: 37272516; NCT04164901).	detail... detail... 37272516
IDH1 R132G	chondrosarcoma	predicted - sensitive	IDH1 Inhibitor	Ivosidenib	Case Reports/Case Series	Actionable	In a Phase I trial, Tibsovo (ivosidenib) treatment was tolerated, substantially decreased plasma 2-HG levels, and resulted in a median progression-free survival of 5.6 months and stable disease in 52% (11/21) of patients with chondrosarcoma harboring IDH1 mutations, including IDH1 R132G (n=3) (PMID: 32208957; NCT02073994).	32208957
IDH1 R132G	acute myeloid leukemia	sensitive	IDH1 Inhibitor	BAY1436032	Preclinical - Patient cell culture	Actionable	In a preclinical study, BAY1436032 decreased (R)-2-hydroxyglutarate (R-2HG) levels and increased differentiation of patient-derived acute myeloid leukemia cells harboring IDH1 R132G in culture (PMID: 28232670).	28232670
IDH1 R132G	Advanced Solid Tumor	predicted - sensitive	IDH1 Inhibitor	DS-1001b	Preclinical - Biochemical	Actionable	In a preclinical study, transformed human cells expressing IDH1 R132G demonstrated sensitivity to DS-1001b in culture, resulting in reduced production of the oncometabolite 2-hydroxyglutarate (2-HG) (PMID: 31727689).	31727689

Showing 1 to 11 of 11 entries

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