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    BIOMARKER:

    IDH1 R132

    i
    Other names: HEL-216, HEL-S-26, Epididymis Luminal Protein 216, Isocitrate Dehydrogenase 1 (NADP+), Epididymis Secretory Protein Li 26, IDH1, Isocitrate Dehydrogenase (NADP(+)) 1, Isocitrate Dehydrogenase 1 (NADP+), Soluble
    Entrez ID:
    3417
    Related biomarkers:
    Expression
    Mutation
    Others
    1d
    IDH1-R132H enhances oncolytic HSV-1 therapy by facilitating viral entry and immune activation in glioma. (PubMed, Nat Commun)
    However, elevated expression of poliovirus receptor (PVR) and the immune checkpoint T-cell immunoreceptor with immunoglobulin and ITIM domain (TIGIT) on tumor-infiltrating leukocytes suggests a potential resistance mechanism to virotherapy. Combining rQNestin34.5 v.2 with TIGIT blockade enhances therapeutic efficacy compared to monotherapy, identifying IDH1-R132H as a potential predictive biomarker for oncolytic virotherapy response.
    Journal • IO biomarker
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IFNG (Interferon, gamma) • TIGIT (T Cell Immunoreceptor With Ig And ITIM Domains 2) • NECTIN1 (Nectin Cell Adhesion Molecule 1)
    |
    IDH1 R132
    |
    linoserpaturev (CAN-3110)
    2d
    A072301: Testing Addition of an Anti-cancer Drug, Vorasidenib to Temozolomide, After Radiation for Advanced Brain Cancer (clinicaltrials.gov)
    P3, N=408, Recruiting, Alliance for Clinical Trials in Oncology | Not yet recruiting --> Recruiting | Initiation date: Oct 2025 --> Jul 2026
    Enrollment open • Trial initiation date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B) • CYP1A2 (Cytochrome P450, family 1, subfamily A, polypeptide 2)
    |
    CDKN2A deletion • IDH1 R132
    |
    temozolomide • Voranigo (vorasidenib)
    3d
    AG120-C-001: Study of Orally Administered AG-120 in Subjects With Advanced Hematologic Malignancies With an IDH1 Mutation (clinicaltrials.gov)
    P1, N=291, Recruiting, Institut de Recherches Internationales Servier | Trial completion date: Mar 2026 --> Dec 2026 | Trial primary completion date: Mar 2026 --> Dec 2026
    Trial completion date • Trial primary completion date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
    |
    IDH1 R132
    |
    Tibsovo (ivosidenib)
    6d
    CPX-351 and Ivosidenib for the Treatment of IDH1 Mutated Acute Myeloid Leukemia or High-Risk Myelodysplastic Syndrome (clinicaltrials.gov)
    P2, N=30, Recruiting, M.D. Anderson Cancer Center | Trial completion date: Jun 2026 --> Jun 2028 | Trial primary completion date: Jun 2026 --> Jun 2028
    Trial completion date • Trial primary completion date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
    |
    IDH1 R132
    |
    Tibsovo (ivosidenib) • Vyxeos (cytarabine/daunorubicin liposomal formulation)
    7d
    Testing the Combination of Anti-cancer Drugs Actimab-A and Cemiplimab (REGN2810) to Improve Outcomes for Patients With Recurrent Glioblastoma (clinicaltrials.gov)
    P1, N=30, Not yet recruiting, National Cancer Institute (NCI) | Initiation date: Jun 2026 --> Dec 2026
    Trial initiation date • Tumor mutational burden • IO biomarker
    |
    PD-L1 (Programmed death ligand 1) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MGMT (6-O-methylguanine-DNA methyltransferase) • CTLA4 (Cytotoxic T-Lymphocyte Associated Protein 4)
    |
    IDH wild-type • IDH1 R132
    |
    Libtayo (cemiplimab-rwlc) • Actimab-A (lintuzumab-Ac225) • Zamyl (lintuzumab)
    7d
    Oncometabolite 2-hydroxyglutarate destabilizes CDH1 to regulate quiescence and enhance chemotherapy sensitivity. (PubMed, Cell Commun Signal)
    Notably, in vitro and in vivo experiments demonstrate that 2-HG induces CDH1 downregulation and reduces quiescent cell populations not only in glioblastoma cells but also in lung cancer and colorectal cancer cells, and consistently sensitizes tumor cells to chemotherapy. Our findings shed light on why IDH1 mutations correlate with better prognosis and highlight the translational potential of 2-HG as a chemotherapy sensitizer.
    Journal
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • CDH1 (Cadherin 1) • CDC20 (Cell Division Cycle 20) • CDK1 (Cyclin-dependent kinase 1)
    |
    IDH1 mutation • IDH1 R132
    11d
    IDH-mutant adult-type diffuse gliomas: a clinicopathological analysis of 1 301 cases (PubMed, Zhonghua Bing Li Xue Za Zhi)
    Compared to supratentorial tumors, non-R132H IDH1 mutations are significantly more frequent in infratentorial tumors. IDH2-mutant gliomas almost exclusively occur in adults and in supratentorial locations, with a significantly higher proportion in oligodendrogliomas than astrocytoma.
    Journal
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
    |
    IDH1 mutation • IDH2 mutation • IDH1 R132 • IDH2 R172
    11d
    Supratentorial papillary glioneuronal tumors: a clinicopathological and genetic analysis of six cases (PubMed, Zhonghua Bing Li Xue Za Zhi)
    Integrated pathologic-molecular analysis is essential for accurately classifying supratentorial papillary glioneuronal tumors. Classic PGNTs are molecularly defined by recurrent PRKCA fusions, while BRAF-altered cases warrant reclassification.
    Journal
    |
    BRAF (B-raf proto-oncogene) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • KIAA1549 • SOX10 (SRY-Box 10) • PRKCA (Protein Kinase C Alpha) • SYP (Synaptophysin) • GFAP (Glial Fibrillary Acidic Protein) • OLIG2 (Oligodendrocyte Transcription Factor 2)
    |
    BRAF V600E • BRAF V600 • BRAF fusion • IDH1 R132
    13d
    VALIENT: VorAsidenib With Lomustine In Patients With rEcurrent IDH-mutaNT Glioma Harboring IDH1 and/or IDH2 Mutations (clinicaltrials.gov)
    P1, N=24, Not yet recruiting, Megan Mantica
    New P1 trial
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
    |
    IDH2 mutation • IDH1 R132 • IDH2 R172
    |
    lomustine • Voranigo (vorasidenib)
    14d
    Phase 3 Study of Vorasidenib (S095032/AG-881) in Asian Participants With Residual or Recurrent Grade 2 Glioma With an IDH1 orIDH2 Mutation (clinicaltrials.gov)
    P3, N=57, Active, not recruiting, Servier | Trial primary completion date: Oct 2025 --> May 2026
    Trial primary completion date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
    |
    IDH2 mutation • IDH1 R132 • IDH2 R172
    |
    Voranigo (vorasidenib)
    14d
    Murine modeling of IDH-mutant 1p/19q-codeleted oligodendroglioma reveals genotype specific phenotypes. (PubMed, bioRxiv)
    Oligo Cdkn2a tumors displayed metabolic and transcriptional changes associated with IDH and CIC mutations, and single cell sequencing identified a bias towards oligodendrocyte differentiation compared to an IDH wild-type glioblastoma mouse model. Oligo Cdkn2a tumors represent the first mouse model system to recapitulate the genetic, histological and transcriptional features of human IDH-mutant 1p/19q-codeleted oligodendrogliomas, offering a platform to further dissect tumor biology and test new therapeutic strategies.
    Preclinical • Journal
    |
    TP53 (Tumor protein P53) • PIK3CA (Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • FUBP1 (Far Upstream Element Binding Protein 1)
    |
    TP53 mutation • IDH wild-type • IDH1 R132
    20d
    Ivosidenib and Combination Chemotherapy for the Treatment of IDH1 Mutant Relapsed or Refractory Acute Myeloid Leukemia (clinicaltrials.gov)
    P1, N=2, Terminated, Northwestern University | Active, not recruiting --> Terminated; Low accrual
    Trial termination
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • UGT1A1 (UDP glucuronosyltransferase family 1 member A1)
    |
    IDH1 R132
    |
    cytarabine • Tibsovo (ivosidenib) • idarubicin hydrochloride • fludarabine IV • Neupogen (filgrastim) • Starasid (cytarabine ocfosfate)