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GENE:

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

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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
1d
CLIP2::MET fusion identifies a molecularly distinct glioneuronal tumor. (PubMed, Discov Oncol)
MET fusions are actionable oncogenic drivers across several cancers, and their detection has therapeutic relevance with approved inhibitors. This case expands the spectrum of MET-driven CNS tumors and suggests that CLIP2::MET fusion may represent a distinct molecular subset of glioneuronal tumors relevant to precision oncology.
Journal
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BRAF (B-raf proto-oncogene) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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BRAF mutation
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
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IFNG (Interferon, gamma) • TIGIT (T Cell Immunoreceptor With Ig And ITIM Domains 2) • NECTIN1 (Nectin Cell Adhesion Molecule 1)
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IDH1 R132
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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
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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)
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CDKN2A deletion • IDH1 R132
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temozolomide • Voranigo (vorasidenib)
2d
Cholangiocarcinoma in the Era of Precision Medicine: Emerging Insights and Therapeutic Strategies. (PubMed, Curr Pharm Des)
Advances in molecular profiling and immunotherapy are reshaping the management of cholangiocarcinoma, enabling more personalized treatment strategies. Continued integration of precision oncology into clinical practice and prospective trials will be critical to improving outcomes for cholangiocarcinoma moving forward.
Journal
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MSI (Microsatellite instability) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • FGFR (Fibroblast Growth Factor Receptor)
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cisplatin • gemcitabine • capecitabine
3d
Trial completion date • Trial primary completion date
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 mutation
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azacitidine • Rezlidhia (olutasidenib)
3d
Recurrent IDH-mutant astrocytoma WHO grade 4 diagnosed during pregnancy: case report with literature review. (PubMed, Front Oncol)
Timely neurosurgical intervention, multidisciplinary care, and integrated obstetric-neurosurgical strategies are crucial. There is a pressing need for clinical guidelines addressing high-grade glioma management in pregnancy, particularly in the era of molecular tumor classification.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B)
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IDH1 mutation • CDKN2A deletion
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
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 R132
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Tibsovo (ivosidenib)
5d
Clinical and Molecular Response to Vorasidenib in Post-Transplant Patient with IDH2-mutant Intrahepatic Cholangiocarcinoma. (PubMed, Oncologist)
This case highlights the potential clinical relevance of IDH-directed therapy in IDH2-mutant cholangiocarcinoma and demonstrates feasibility in an immunosuppressed post-transplant setting. These findings are hypothesis-generating and support further evaluation of IDH inhibition in this population.
Journal • IO biomarker
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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IDH2 mutation • IDH2 R172
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Voranigo (vorasidenib)
6d
A Phase Ib Trial of Azacitidine, Venetoclax and Allogeneic NK Cells for Acute Myeloid Leukemia (ADVENT-AML) (clinicaltrials.gov)
P1, N=32, Recruiting, M.D. Anderson Cancer Center | Trial primary completion date: Jun 2026 --> Jun 2028
Trial primary completion date
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FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • BCL2 (B-cell CLL/lymphoma 2) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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Venclexta (venetoclax) • azacitidine
6d
Advances in Brain Tumor Biomarkers: From Molecular Profiling to Liquid Biopsy and AI-Driven Detection. (PubMed, Cancers (Basel))
In parallel, artificial intelligence and machine learning are advancing the field by integrating multi-omics and radiomic data to enhance detection, classify tumors, and predict key molecular alterations, supporting the emerging framework of radiogenomics. Together, these developments are driving a shift toward more precise and dynamic approaches to brain tumor diagnosis and management, with relevance for improving outcomes in older adults with brain cancer.
Review • Journal • Liquid biopsy
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MGMT (6-O-methylguanine-DNA methyltransferase) • TERT (Telomerase Reverse Transcriptase)
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MGMT promoter methylation
6d
Cytogenetic and Molecular Analysis of a "Double-Hit" RUNX1 Including a RUNX1 p.Trp279* and a Cryptic Novel t(6;21)(q25;q22)/RUNX1::ARID1B in Acute Myeloid Leukemia. (PubMed, Genes Chromosomes Cancer)
This study expands the spectrum of RUNX1 fusions and highlights the integral diagnostic value of morphology, flow cytometry, cytogenetics, FISH, and NGS analyses for broad structural variant detection in clinical practice. Furthermore, the truncating mutation in one allele of RUNX1 and RUNX1::ARID1B of the second allele detected with advanced disease suggests the possibility of combined transcriptional and chromatin regulatory alterations in disease recurrence in the patient.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • RUNX1 (RUNX Family Transcription Factor 1) • ARID1B (AT-Rich Interaction Domain 1B) • CD34 (CD34 molecule) • PTPRC (Protein Tyrosine Phosphatase Receptor Type C)
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IDH1 mutation
6d
IDH1-Associated m6A Methylation Is Linked to Transcriptomic Heterogeneity in Glioma. (PubMed, Cancers (Basel))
Overall, these data describe subtype-specific patterns of m6A marking and isoform architecture across glioma tissues, derived from computational inference using direct RNA sequencing in a modestly sized cohort and warrant validation by orthogonal methods in larger studies. These findings are consistent with concurrent independent evidence that isoform-specific m6A deposition is evolutionarily conserved across mammals and that long-read isoform resolution reveals transcript diversity in glioma not captured by gene-level analysis. While cohort size and the absence of orthogonal site-level validation suggest that the data require cautious interpretation, this work provides a hypothesis-generating resource and methodological framework for future mechanistic and translational investigation of the glioma epitranscriptome.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 mutation • IDH wild-type