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Profile Name IDH1 R132H
Gene Variant Detail

IDH1 R132H (gain of function)

Relevant Treatment Approaches IDH Inhibitor (Pan) IDH1 Inhibitor IDH1 R132H peptide vaccine PEPIDH1M vaccine

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Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
FLT3 D835Y IDH1 R132H hematologic cancer resistant AGI-5198 Preclinical - Cell culture Actionable In a preclinical study, AGI-5198 treatment did not decrease proliferation of transformed cells expressing FLT3 D835Y and IDH1 R132H in culture (PMID: 30651561). 30651561
FLT3 D835Y IDH1 R132H hematologic cancer sensitive Crenolanib Preclinical - Cell culture Actionable In a preclinical study, Crenolanib (CP-868596) decreased proliferation of transformed cells expressing FLT3 D835Y and IDH1 R132H in culture (PMID: 30651561). 30651561
FLT3 D835Y IDH1 R132H hematologic cancer sensitive AGI-5198 + Crenolanib Preclinical - Cell culture Actionable In a preclinical study, the combination of Crenolanib (CP-868596) and AGI-5198 synergistically decreased proliferation of transformed cells expressing FLT3 D835Y and IDH1 R132H in culture (PMID: 30651561). 30651561
FLT3 exon 14 ins IDH1 R132H hematologic cancer sensitive Crenolanib Preclinical - Cell culture Actionable In a preclinical study, Crenolanib (CP-868596) decreased proliferation of transformed cells expressing FLT3-ITD and IDH1 R132H in culture (PMID: 30651561). 30651561
FLT3 exon 14 ins IDH1 R132H hematologic cancer sensitive AGI-5198 + Crenolanib Preclinical - Cell culture Actionable In a preclinical study, transformed cells expressing FLT3-ITD and IDH1 R132H demonstrated increased sensitivity to treatment with the combination of Crenolanib (CP-868596) and AGI-5198 compared to treatment with Crenolanib (CP-868596) alone in culture (PMID: 30651561). 30651561
IDH1 R132H IDH1 D279N acute myeloid leukemia predicted - resistant Ivosidenib Preclinical - Cell culture Actionable In a preclinical study, Tibsovo (ivosidenib) modestly suppressed (R)-2-hydroxyglutarate (R-2HG) production in an acute myeloid leukemia cell line expressing IDH1 R132H and D279N but did not inhibit cytokine-independent growth in culture (PMID: 36056177). 36056177
IDH1 R132H IDH1 D279N acute myeloid leukemia sensitive LY3410738 Preclinical - Cell culture Actionable In a preclinical study, LY3410738 inhibited (R)-2-hydroxyglutarate (R-2HG) production and cytokine-independent growth in an acute myeloid leukemia cell line expressing IDH1 R132H and D279N in culture (PMID: 36056177). 36056177
IDH1 R132H IDH1 S280F leukemia predicted - resistant Ivosidenib Preclinical - Biochemical Actionable In a preclinical study, Tibsovo (ivosidenib) did not inhibit Idh1 activity and R-2HG production in a leukemia cell line expressing IDH1 R132H and S280F in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 H315D leukemia resistant Ivosidenib Preclinical - Cell line xenograft Actionable In a preclinical study, Tibsovo (ivosidenib) did not inhibit Idh1 activity and R-2HG production in a leukemia cell line expressing IDH1 R132H and H315D in culture and did not reduce tumor burden in a cell line xenograft model (PMID: 36222845). 36222845
IDH1 R132H IDH1 H315D leukemia predicted - resistant AG-881 Preclinical - Biochemical Actionable In a preclinical study, Vorasidenib (AG-881) did not inhibit R-2HG production in a leukemia cell line expressing IDH1 R132H and H315D in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 H315D leukemia predicted - resistant IDH305 Preclinical - Biochemical Actionable In a preclinical study, IDH305 did not inhibit R-2HG production in a leukemia cell line expressing IDH1 R132H and H315D in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 H315D leukemia predicted - resistant BAY1436032 Preclinical - Biochemical Actionable In a preclinical study, BAY1436032 did not inhibit R-2HG production in a leukemia cell line expressing IDH1 R132H and H315D in culture (PMID: 36222845). 36222845
IDH1 R132H IDH2 R140Q acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a Phase I trial, IDH2 R140Q was identified at progression in 4 patients with acute myeloid leukemia harboring IDH1 R132H who previously responded to Tibsovo (ivosidenib) treatment (PMID: 32380538; NCT02074839). 32380538
IDH1 R132H IDH1 T313I leukemia resistant Ivosidenib Preclinical - Cell line xenograft Actionable In a preclinical study, Tibsovo (ivosidenib) did not inhibit Idh1 activity and R-2HG production in a leukemia cell line expressing IDH1 R132H and T313I in culture and did not reduce tumor burden in a cell line xenograft model (PMID: 36222845). 36222845
IDH1 R132H IDH1 T313I leukemia predicted - resistant AG-881 Preclinical - Biochemical Actionable In a preclinical study, Vorasidenib (AG-881) did not inhibit R-2HG production in a leukemia cell line expressing IDH1 R132H and T313I in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 T313I leukemia predicted - resistant IDH305 Preclinical - Biochemical Actionable In a preclinical study, IDH305 did not inhibit R-2HG production in a leukemia cell line expressing IDH1 R132H and T313I in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 T313I leukemia predicted - resistant BAY1436032 Preclinical - Biochemical Actionable In a preclinical study, BAY1436032 did not inhibit R-2HG production in a leukemia cell line expressing IDH1 R132H and T313I in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 L409V leukemia predicted - sensitive Ivosidenib Preclinical - Biochemical Actionable In a preclinical study, Tibsovo (ivosidenib) inhibited enzymatic activity and R-2HG production in a leukemia cell line expressing IDH1 R132H and L409V in culture (PMID: 36222845). 36222845
IDH1 R132H IDH1 A410T leukemia predicted - sensitive Ivosidenib Preclinical - Biochemical Actionable In a preclinical study, Tibsovo (ivosidenib) inhibited enzymatic activity and R-2HG production in a leukemia cell line expressing IDH1 R132H and A410T in culture (PMID: 36222845). 36222845