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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
17h
New P2 trial • Head-to-Head • IO biomarker
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1)
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IDH2 mutation • NPM1 mutation
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Venclexta (venetoclax) • cytarabine • azacitidine • daunorubicin
19h
Diffuse Intraductal Breast Papillomatosis in a 34-Year-Old Female With Maffucci Syndrome: A Case Report. (PubMed, Case Rep Radiol)
This case raises the possibility of an association between Maffucci syndrome and recurrent papillary breast lesions. In the absence of molecular confirmation, this relationship remains speculative and should be regarded as hypothesis generating, underscoring the need for further investigation with genetic and immunohistochemical correlation rather than changes to established breast cancer screening guidelines.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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IDH1 mutation • IDH2 mutation • IDH2 R172
23h
Enrollment open
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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IDH wild-type
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Zynyz (retifanlimab-dlwr)
2d
Long-term remission and survival in older adults with IDH-mutated acute myeloid leukemia treated with IDH inhibitors. (PubMed, Leuk Res)
This selected real-world cohort demonstrates durable responses exceeding published benchmarks and has one of the longest follow-up periods reported for IDHi in AML. A subset of older adults with IDH-mutated AML can achieve highly durable remissions on IDHi without significant toxicity.
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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Tibsovo (ivosidenib) • Idhifa (enasidenib)
2d
18F-NaF PET/CT Versus 18F-FDG PET/CT for Baseline Mapping in Ollier Disease: A Pediatric Case. (PubMed, J Nucl Med Technol)
18F-FDG PET/CT shows limited sensitivity because of low glycolytic activity and physiologic uptake. This case highlights the superior performance of 18F-NaF PET/CT for osteoblastic lesion detection and anatomic delineation in a pediatric patient with IDH1-mutated Ollier disease.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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IDH1 mutation
3d
A Study of CD371-YSNVZIL-18 CAR T Cells in People With Acute Myeloid Leukemia (clinicaltrials.gov)
P1, N=15, Recruiting, Memorial Sloan Kettering Cancer Center | Trial completion date: Aug 2026 --> Dec 2026 | Trial primary completion date: Aug 2026 --> Dec 2026
Trial completion date • Trial primary completion date
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FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • IL18 (Interleukin 18) • CD37 (CD37 Molecule)
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FLT3-ITD mutation • IDH1 mutation • IDH2 mutation
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CD371-YSNVZ-IL18 CAR T cells
3d
Reductive carboxylation via isocitrate dehydrogenase 1 supports cardiac metabolic adaptation during oncometabolic stress. (PubMed, bioRxiv)
Lastly, epigenetic analyses of histone marks revealed that IDH1 induces widespread alterations in histone acetylation and tri-methylation. Our results highlight a novel role for IDH1 in cardiac metabolism and transcriptional control of metabolic adaptation to tumor-mediated stress and provide evidence that reductive-citrate formation may induce epigenetic modifications in the heart.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
6d
Insight into the metabolism of gliomas by MRI and MRS. (PubMed, Biophys Rev)
Further, multi-center clinical trials and incorporating metabolic endpoints are necessary for adaptive therapy strategies. Together, MRI and MRS provide a comprehensive view of glioma biology that supports precision diagnosis, risk stratification, and individualized treatment planning, thereby advancing the goal of routine clinical adoption of metabolic imaging in neuro-oncology.
Review • Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • TERT (Telomerase Reverse Transcriptase) • ATRX (ATRX Chromatin Remodeler)
7d
Case Report: Response to ivosidenib in patients with cholangiocarcinoma: a clinical perspective with illustrative cases. (PubMed, Front Oncol)
Performing molecular testing (in the absence of adequate tumor tissue with liquid biopsy) as early as possible is already an integral part of the treatment pathway planning for CCA patients. Early molecular testing may therefore lead to the possibility of administering targeted therapy in the first-line setting within this patient group, pending the outcomes of clinical trials.
Journal
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HER-2 (Human epidermal growth factor receptor 2) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1)
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EGFR mutation • HER-2 mutation • IDH1 mutation
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Tibsovo (ivosidenib)
8d
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
8d
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)
9d
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)