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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
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SOX10 distinguishes oligodendrogliomas from low-grade glioneuronal tumors/low-grade gliomas with oligodendrocyte-like features. (PubMed, J Pathol Clin Res)
In conclusion, SOX10 negativity effectively excludes low-grade glioneuronal tumors/low-grade gliomas with oligodendrocyte-like features (except PLNTY) and non-neoplastic oligodendrocyte hyperplasia from oligodendrogliomas. SOX10 serves as a specific diagnostic marker, enhancing pathological diagnostic accuracy.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • SOX10 (SRY-Box 10) • OLIG2 (Oligodendrocyte Transcription Factor 2)
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Isocitrate Dehydrogenase-1 Mutation Status and Dynamic Subcortical Motor Mapping Using Ultrasonic Aspirator Stimulation. (PubMed, Oper Neurosurg)
The interpretability of distance-based subcortical motor mapping is influenced by IDH1 mutation status, whereas continuous CUSA stimulation and ball-tip stimulation show comparable overall distance-threshold behavior. Distance-based scMEP thresholds are more reliable in IDH1-mutant tumors, whereas increased variability in IDH1-wildtype tumors likely reflects greater susceptibility to intraoperative brain shift and other biological factors, supporting a biologically informed, real-time electrophysiological approach in motor-eloquent tumor resections.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 mutation • IDH wild-type
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IDH1-mutant vaccine in newly diagnosed astrocytoma: final analysis of the multicenter, single-arm, open-label, first-in-human phase 1 NOA16 trial. (PubMed, Nat Cancer)
IDH1-vac-induced T cell responses were detected in the inflamed brain lesion of an IDH1-vac-associated pseudoprogression, whereas no IDH1-vac-induced T cells were found in participants with early progressive disease. The favorable long-term outcome of the NOA16 cohort supports investigating IDH1-vac in persons with newly diagnosed grade 3 and 4 (World Health Organization classification 2021) IDH-mutant astrocytomas in a randomized phase 2 trial (ClinicalTrials.gov identifier: NCT02454634 ).
P1 data • Journal • First-in-human
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 mutation • IDH1 R132
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Immune cell landscape reveals 5 immune-related subtypes and molecular characteristics with prognostic and therapeutic implications in pan-cancer. (PubMed, J Leukoc Biol)
These findings highlight the heterogeneity of immune cell infiltration in tumors and its impact on patient prognosis and treatment sensitivity. The findings suggest that immune subtypes can serve as valuable biomarkers, particularly IDH1 mutations in cold tumors, paving the way for personalized cancer therapies.
Journal • IO biomarker • Pan tumor
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 mutation
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Advanced cholangiocarcinoma in 2025: Therapeutic sequencing and global implementation. (PubMed, Med)
Chemoimmunotherapy is the reference first-line regimen for biomarker-unselected advanced cholangiocarcinoma. Early comprehensive genomic profiling is essential to enable timely, matched targeted therapy and maximize population-level benefit.
Journal • MSi-H Biomarker • PD(L)-1 Biomarker • IO biomarker
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HER-2 (Human epidermal growth factor receptor 2) • MSI (Microsatellite instability) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • FGFR (Fibroblast Growth Factor Receptor)
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MSI-H/dMMR • IDH1 mutation • EGFR positive
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Keytruda (pembrolizumab) • cisplatin • Imfinzi (durvalumab) • gemcitabine • 5-fluorouracil • Enhertu (fam-trastuzumab deruxtecan-nxki) • oxaliplatin • Tibsovo (ivosidenib) • leucovorin calcium
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Enrollment closed
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • MGMT (6-O-methylguanine-DNA methyltransferase)
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IDH1 R132 • IDH wild-type
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temozolomide • peposertib (M3814)
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Targeted Therapies Combined with Intensive Chemotherapy in Fit Acute Myeloid Leukemia: Past Developments, Current Evidence, and Future Therapeutic Paradigms. (PubMed, J Clin Med)
We discuss the biological rationale for combination approaches and summarize the key clinical studies that have defined current practice, including trials evaluating FLT3 inhibitors, gemtuzumab ozogamicin, IDH inhibitors, and venetoclax-based strategies. While genotype-directed strategies-such as FLT3 inhibition-have already demonstrated survival benefit, optimal patient selection, treatment sequencing, and duration remain areas of active investigation. Future progress will likely depend on MRD-driven treatment adaptation, improved understanding of clonal evolution, and the development of rational multi-agent combinations capable of achieving deeper and more durable remissions.
Review • Journal
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FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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Venclexta (venetoclax) • Mylotarg (gemtuzumab ozogamicin)
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The Role of AXL Signaling and Mutant Isocitrate Dehydrogenase 1/2 in Conventional Chondrosarcoma. (PubMed, Cancers (Basel))
This review synthesizes current evidence on the roles of IDH1/2 mutations and dysregulation of AXL signaling in CS, emphasizing their potential contributions to tumor aggressiveness, immune suppression, and resistance to therapy. Additionally, we explore current developments in targeted therapy exploiting IDH1/2 and AXL dysregulation.
Review • Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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IDH1 mutation • IDH2 mutation
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The Evolving Landscape of Immune Regulation and Immunotherapy in Cholangiocarcinoma and Biliary Tract Cancer. (PubMed, Cancers (Basel))
We evaluate the expanding clinical trial landscape of immunotherapy in CCA and more broadly in BTC, including adoptive cell therapies and cancer vaccines. Together, these advances position CCA as a paradigm of how tumor genotype and microenvironment co-evolve to define immunotherapy sensitivity and resistance.
Review • Journal • IO biomarker
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KRAS (KRAS proto-oncogene GTPase) • FGFR2 (Fibroblast growth factor receptor 2) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • MTAP (Methylthioadenosine Phosphorylase)
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KRAS mutation • IDH1 mutation • FGFR2 mutation • FGFR2 fusion
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Gadolinium-Free MR Perfusion Imaging Based on Generative Adversarial Network for Primary Intracranial Tumor Diagnosis: A Multi-Center Study. (PubMed, Neuro Oncol)
As a gadolinium-free alternative, CBVsyn maps synthesized by TA-GAN correlates with pathologic findings and demonstrates promise for characterizing primary intracranial tumors.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • ENG (Endoglin)
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Molecular and Clinical Determinants of Targeted Therapy Treatment in Biliary Tract Cancer. (PubMed, Clin Cancer Res)
This comprehensive molecular profiling study illustrates the real-world utility and limitations of targeted next-generation sequencing of BTC and affirms the use of precision medicine in patients with these diseases. Characterization of genomic heterogeneity and therapeutic resistance has the potential to inform ongoing drug development efforts for BTC.
Journal
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HER-2 (Human epidermal growth factor receptor 2) • KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • MET (MET proto-oncogene, receptor tyrosine kinase) • FGFR2 (Fibroblast growth factor receptor 2) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • MTAP (Methylthioadenosine Phosphorylase) • MDM2 (E3 ubiquitin protein ligase) • SMAD4 (SMAD family member 4) • NTRK (Neurotrophic receptor tyrosine kinase)
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MET amplification • MTAP deletion