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    GENE:

    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)

    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
    19h
    NRG-BN011: Testing the Addition of the Chemotherapy Drug Lomustine (Gleostine) to the Usual Treatment (Temozolomide and Radiation Therapy) for Newly Diagnosed MGMT Methylated Glioblastoma (clinicaltrials.gov)
    P3, N=306, Suspended, NRG Oncology | Recruiting --> Suspended
    Trial suspension
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • MGMT (6-O-methylguanine-DNA methyltransferase)
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    MGMT promoter methylation • IDH1 R132
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    temozolomide • lomustine
    1d
    Cerebrospinal fluid cfDNA-based molecular assessment of resection extent and prognosis in glioma. (PubMed, Commun Med (Lond))
    These findings suggest that CSF cfDNA effectively represents the genetic profile of gliomas and serves as a sensitive measure for surgical resection efficacy and patient prognosis, highlighting its potential as a non-invasive biomarker for enhancing post-operative management in glioma patients.
    Journal • Tumor mutational burden
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    TP53 (Tumor protein P53) • TMB (Tumor Mutational Burden) • PTEN (Phosphatase and tensin homolog) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • TERT (Telomerase Reverse Transcriptase)
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    TP53 mutation • PTEN mutation
    1d
    18F-DOPA-PET and advanced MRI improve treatment response assessment in IDH1/2-mutant gliomas treated with IDH inhibitors. (PubMed, Clin Cancer Res)
    These results highlight the potential of ¹⁸F-DOPA-PET and advanced MRI sequences as valuable complements to standard RANO 2.0 MRI evaluations for assessing treatment response in glioma patients undergoing IDHi therapy.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    Tibsovo (ivosidenib) • Voranigo (vorasidenib)
    1d
    Exploring CSF microRNA signatures as diagnostic biomarkers in adult-type diffuse gliomas. (PubMed, Noncoding RNA Res)
    Ingenuity Pathway Analysis (IPA) revealed that miR-16-5p and other miRNAs with seed AGCAGCA formed the largest interaction network in GBM, while disease enrichment analysis using Database for Annotation, Visualization, and Integrated Discovery (DAVID) confirmed that the 1000 predicted mRNA targets of DE-miRNAs in GBM were disease relevant. Collectively, these findings identify a robust panel of CSF-derived miRNAs capable of distinguishing IDH-mutant gliomas, GBM, and non-tumor states, supporting the potential of EV-miRNAs as minimally invasive biomarkers for the molecular characterization of diffuse gliomas.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MIR21 (MicroRNA 21) • MIR142 (MicroRNA 142) • MIR16 (MicroRNA 16) • MIR19B1 (MicroRNA 19b-1) • MIR27B (MicroRNA 27b) • MIR99A (MicroRNA 99a) • MIR150 (MicroRNA 150) • MIR195 (MicroRNA 195) • MIR26A1 (MicroRNA 26a-1) • MIR30A (MicroRNA 30a) • MIR30E (MicroRNA 30e)
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    IDH wild-type
    2d
    Non-coding small RNAs Buffer protein interactions to prevent oncogenic aggregation: structural dampening of aberrant PPIs by RNA. (PubMed, RNA Biol)
    At the network level, physiological PPIs exhibit high shared ncRNA buffering capacity, whereas oncogenic interactions are characterized by reduced or absent RNA overlap. AlphaFold3 modelling of mutant IDH1/2 complexes illustrates how loss of RNA buffering permits excessive stabilization of enzyme-associated interfaces, reflected by directional changes in buried surface area (ΔBSA) and contact heterogeneity.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation
    3d
    A Real-World Study of Precision Treatment for Advanced Cholangiocarcinoma Based on Molecular Subtyping (ChiCTR2500111407)
    P=N/A, N=55, Not yet recruiting, Fuzhou University Affiliated Provincial Hospital; Fuzhou University Affiliated Provincial Hospital
    New trial
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    EGFR (Epidermal growth factor receptor) • HER-2 (Human epidermal growth factor receptor 2) • KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • PIK3CA (Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) • MET (MET proto-oncogene, receptor tyrosine kinase) • BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • MSI (Microsatellite instability) • RET (Ret Proto-Oncogene) • FGFR2 (Fibroblast growth factor receptor 2) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • NRG1 (Neuregulin 1) • VEGFA (Vascular endothelial growth factor A) • RNF43 (Ring Finger Protein 43) • TYK2 (Tyrosine Kinase 2) • NTRK (Neurotrophic receptor tyrosine kinase)
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    BRAF V600E • KRAS mutation • EGFR mutation • MSI-H/dMMR • HER-2 overexpression • HER-2 amplification • PIK3CA mutation • BRAF V600 • MET amplification • RET fusion • FGFR2 mutation • PALB2 mutation • FGFR2 fusion • MET mutation • IDH mutation + BRAF V600E • IDH mutation + NTRK fusion
    4d
    Bile duct tumor thrombus (intraductal polypoid growth)-positive intrahepatic cholangiocarcinoma: clinicopathologic and genomic analysis. (PubMed, J Pathol)
    These results highlight the importance of evaluating BDTT in SDT, as it may be the main route of hilar extension in aggressive cases.
    Journal
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    KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • TP53 (Tumor protein P53) • PIK3CA (Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) • NRAS (Neuroblastoma RAS viral oncogene homolog) • FGFR2 (Fibroblast growth factor receptor 2) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • ARID1A (AT-rich interaction domain 1A) • NF1 (Neurofibromin 1) • BAP1 (BRCA1 Associated Protein 1) • KMT2D (Lysine Methyltransferase 2D) • MUC1 (Mucin 1) • SMAD4 (SMAD family member 4) • MLL2 (Myeloid/lymphoid or mixed-lineage leukemia 2) • TGFBR2 (Transforming Growth Factor Beta Receptor 2) • MUC4 (Mucin 4, Cell Surface Associated) • CDX2 (Caudal Type Homeobox 2) • MUC2 (Mucin 2) • RSPO3 (R-Spondin 3) • CACNA1A (Calcium Voltage-Gated Channel Subunit Alpha1 A) • MUC17 (Mucin 17) • MUC5AC (Mucin 5AC) • MUC6 (Mucin 6) • PTPRK (Protein Tyrosine Phosphatase Receptor Type K) • TGFBR1 (Transforming Growth Factor Beta Receptor 1)
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    FGFR2 mutation • FGFR2 fusion • FGFR2 rearrangement
    5d
    Oxidative Phosphorylation in Silent Pituitary Adenomas: A Multiomics Perspective. (PubMed, Int J Endocrinol)
    These findings suggest that the oxidative phosphorylation pathway plays a pivotal role in the secretory functions of pituitary adenomas. This study offers new insights into the biological mechanisms underlying pituitary adenomas and provides valuable directions for future research, emphasizing the importance of oxidative phosphorylation in tumor behavior and potential therapeutic targets.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • MAPK1 (Mitogen-activated protein kinase 1) • MAPK3 (Mitogen-Activated Protein Kinase 3) • TCHH (Trichohyalin)
    5d
    Olutasidenib in recurrent/relapsed locally advanced or metastatic IDH1-mutated chondrosarcoma: phase 1b/2 trial. (PubMed, Nat Commun)
    Olutasidenib was well tolerated and conferred disease control in cCS. Study limitations include open-label design and low patient sample due to rarity of cCS.
    P1/2 data • Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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    IDH1 mutation
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    Rezlidhia (olutasidenib)
    6d
    Repeatability of Magnetic Resonance Imaging in Patients With IDH1 Mutant Glioma on Ivosidenib (clinicaltrials.gov)
    P=N/A, N=0, Withdrawn, Duke University | N=40 --> 0 | Not yet recruiting --> Withdrawn
    Enrollment change • Trial withdrawal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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    IDH1 mutation
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    Tibsovo (ivosidenib)
    7d
    Current Pharmacotherapeutic Strategies in Diffuse Gliomas: Focus on Glioblastoma, IDH-Wildtype, and Emerging Targeted Therapies for IDH-Mutant Tumors. (PubMed, Pharmaceuticals (Basel))
    This review focuses on contemporary pharmacotherapeutic approaches used in the management of glioblastoma, IDH-wildtype, including temozolomide-based chemotherapy, corticosteroids for edema control, and antiangiogenic therapy in recurrent disease, with particular emphasis on their clinical efficacy and limitations...Particular attention is given to ivosidenib, a selective inhibitor of mutant IDH1, currently evaluated for the treatment of astrocytoma, IDH-mutant, grade 4...Finally, innovative drug-delivery technologies designed to overcome the blood-brain barrier are briefly discussed as complementary strategies that may enhance the efficacy of both conventional and targeted therapies. Overall, future advances in the treatment of diffuse gliomas will likely depend on the integration of molecularly targeted agents, predictive biomarkers, and advanced delivery platforms aimed at improving patient survival and quality of life.
    Review • Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation • IDH wild-type
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    temozolomide • Tibsovo (ivosidenib)
    7d
    Evaluation of the Idylla IDH1-2 Mutation Assay for the Detection of IDH Variants in Solid Tumors and Hematological Malignancies. (PubMed, Int J Mol Sci)
    Based on these data, the Idylla IDH1-2 mutation assay represents a fast and reliable alternative to detect IDH hotspot variants in solid tumors and hematological malignancies using either fixed tissue sections or DNA extracts. Particular attention, however, is needed for the interpretation of cases with cycle of quantification values of the internal controls over 35, for which a variant with low allelic frequency could be missed due to low DNA quantity or quality.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 R132 • IDH2 R172
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    Idylla™ IDH1-2 Mutation Assay