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GENE:

IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)

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Other names: IDH2, Isocitrate Dehydrogenase (NADP(+)) 2, Isocitrate Dehydrogenase (NADP(+)) 2, Mitochondrial, Isocitrate Dehydrogenase 2 (NADP+), Mitochondrial, Isocitrate Dehydrogenase [NADP], Mitochondrial, Oxalosuccinate Decarboxylase, NADP(+)-Specific ICDH, ICD-M, IDH, IDP, MNADP-IDH, D2HGA2, IDHM, IDPM
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
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
Posttranscriptional Regulation of Metabolism in Glioblastoma: A Multipathway Review. (PubMed, J Biochem Mol Toxicol)
We also discuss therapeutic strategies targeting these miRNA-metabolism circuits, including nanoparticle delivery, dietary restriction, and combination therapies that re-sensitize tumors to temozolomide and radiation. Understanding and therapeutically exploiting these networks presents a powerful approach to overcoming GBM's metabolic resilience, thereby opening new avenues for precision oncology.
Review • Journal
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IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • CREBBP (CREB binding protein) • MIR200B (MicroRNA 200b) • MIR126 (MicroRNA 126) • MIR375 (MicroRNA 375) • MIR183 (MicroRNA 183) • SLC2A1 (Solute Carrier Family 2 Member 1) • XIST (X Inactive Specific Transcript)
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temozolomide
3d
Triple metachronous primary brain tumors: sequential occurrence of astrocytoma, atypical meningioma, and high-grade glioma without radiotherapy exposure. Illustrative case. (PubMed, J Neurosurg Case Lessons)
This case underscores that metachronous collision tumors can arise without radiotherapy, necessitating histopathological and molecular integration for accurate diagnosis. Long-term vigilance is critical for detecting sequential tumors, and shared microenvironments or genetic pathways may underlie tumorigenesis. Comprehensive profiling aids in clarifying pathogenesis and guiding management. https://thejns.org/doi/10.3171/CASE26149.
Journal
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IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NOTCH1 (Notch 1) • NCOR2 (Nuclear Receptor Corepressor 2) • CHD4 (Chromodomain Helicase DNA Binding Protein 4) • GFAP (Glial Fibrillary Acidic Protein)
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IDH2 R172
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
Trial initiation date • Tumor mutational burden • IO biomarker
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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)
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IDH wild-type • IDH1 R132
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Libtayo (cemiplimab-rwlc) • Actimab-A (lintuzumab-Ac225) • Zamyl (lintuzumab)
8d
Mitochondrial bioenergetic signatures differentiate asymptomatic from symptomatic Alzheimer's disease. (PubMed, Commun Biol)
In contrast, microglia and oligodendrocytes proportions were reduced in AsymAD relative to AD. Our findings identify preserved mitochondrial bioenergetics in AsymAD and suggest that enhancing NADH metabolism via NAD+ precursor-based interventions may potentially help in maintaining cognitive function despite amyloid and tau pathology.
Journal
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IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MB (Myoglobin) • MRPL47 (Mitochondrial Ribosomal Protein L47)
10d
Prognostic impact of variant allele frequency in intensively treated patients with NPM1-mutated AML: a PETHEMA study. (PubMed, Blood)
Notably, intra-clonal co-localization of NPM1 with IDH1 or TET2 was associated with improved outcomes, whereas co-localization with WT1 predicted dismal prognosis. These results demonstrate that quantitative and structural dimensions of clonality refine the biological and prognostic landscape of NPM1-mutated AML beyond mutational status alone.
Journal • Tumor mutational burden
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KRAS (KRAS proto-oncogene GTPase) • TMB (Tumor Mutational Burden) • FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1) • DNMT3A (DNA methyltransferase 1) • TET2 (Tet Methylcytosine Dioxygenase 2) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • WT1 (WT1 Transcription Factor)
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NPM1 mutation
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
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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IDH1 mutation • IDH2 mutation • IDH1 R132 • IDH2 R172
12d
Mutational Landscape and Clinical Outcomes in AML With Sole Trisomy 8. (PubMed, Hematol Oncol)
Categorizing patients on the basis of MR gene mutations revealed that the inferior survival of sole +8 patients may be attributed to the high frequency of MR gene mutations in these patients. These findings indicate the importance of genetic mutations, specifically MR genes, in sole +8 AML.
Clinical data • Journal
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FLT3 (Fms-related tyrosine kinase 3) • NRAS (Neuroblastoma RAS viral oncogene homolog) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • RUNX1 (RUNX Family Transcription Factor 1) • ASXL1 (ASXL Transcriptional Regulator 1) • TET2 (Tet Methylcytosine Dioxygenase 2) • SRSF2 (Serine and arginine rich splicing factor 2) • STAG2 (Stromal Antigen 2)
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ASXL1 mutation • TET2 mutation • SRSF2 mutation