IDH Mutations in Glioma: Double-Edged Sword in Clinical Applications?

Biomedicines - Tập 9 Số 7 - Trang 799
Alişan Kayabölen1,2, Ebru Yilmaz1,2, Tugba Bagcı-Önder1,2
1Brain Cancer Research and Therapy Lab, Koç University School of Medicine, 34450 Istanbul, Turkey
2**Koç University Research Center for Translational Medicine (KUTTAM), 34450 Istanbul, Turkey

Tóm tắt

Discovery of point mutations in the genes encoding isocitrate dehydrogenases (IDH) in gliomas about a decade ago has challenged our view of the role of metabolism in tumor progression and provided a new stratification strategy for malignant gliomas. IDH enzymes catalyze the conversion of isocitrate to alpha-ketoglutarate (α-KG), an intermediate in the citric acid cycle. Specific mutations in the genes encoding IDHs cause neomorphic enzymatic activity that produces D-2-hydroxyglutarate (2-HG) and result in the inhibition of α-KG-dependent enzymes such as histone and DNA demethylases. Thus, chromatin structure and gene expression profiles in IDH-mutant gliomas appear to be different from those in IDH-wildtype gliomas. IDH mutations are highly common in lower grade gliomas (LGG) and secondary glioblastomas, and they are among the earliest genetic events driving tumorigenesis. Therefore, inhibition of mutant IDH enzymes in LGGs is widely accepted as an attractive therapeutic strategy. On the other hand, the metabolic consequences derived from IDH mutations lead to selective vulnerabilities within tumor cells, making them more sensitive to several therapeutic interventions. Therefore, instead of shutting down mutant IDH enzymes, exploiting the selective vulnerabilities caused by them might be another attractive and promising strategy. Here, we review therapeutic options and summarize current preclinical and clinical studies on IDH-mutant gliomas.

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Tài liệu tham khảo

Ostrom, 2016, CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2009–2013, Neuro-Oncology, 18, v1, 10.1093/neuonc/now207

Louis, 2016, The 2016 World Health Organization Classification of Tumors of the Central Nervous System: A summary, Acta Neuropathol., 131, 803, 10.1007/s00401-016-1545-1

Bowman, 2017, GlioVis data portal for visualization and analysis of brain tumor expression datasets, Neuro-Oncology, 19, 139, 10.1093/neuonc/now247

Hurley, 1991, Catalytic Mechanism of NADP+-Dependent Isocitrate Dehydrogenase: Implications from the Structures of Magnesium-Isocitrate and NADP + Complexes, Biochemistry, 30, 8671, 10.1021/bi00099a026

Lee, 2002, Cytosolic NADP+-dependent isocitrate dehydrogenase status modulates oxidative damage to cells, Free Radic. Biol. Med., 32, 1185, 10.1016/S0891-5849(02)00815-8

Losman, 2013, What a difference a hydroxyl makes: Mutant IDH, (R)-2-hydroxyglutarate, and cancer, Genes Dev., 27, 836, 10.1101/gad.217406.113

Ramachandran, 1980, Chemical characterization of distinct subunits of pig heart DPN-specific isocitrate dehy-drogenase, J. Biol. Chem., 255, 8859, 10.1016/S0021-9258(18)43581-8

Xu, 2004, Structures of human cytosolic NADP-dependent isocitrate dehydrogenase reveal a novel self-regulatory mechanism of activity, J. Biol. Chem., 279, 33946, 10.1074/jbc.M404298200

Gabriel, 1986, Activity of purified NAD-specific isocitrate dehydrogenase at modulator and sub-strate concentrations approximating conditions in mitochondria, Metabolism, 35, 661, 10.1016/0026-0495(86)90175-7

Hartmann, 2009, Type and frequency of IDH1 and IDH2 mutations are related to astrocytic and oligodendroglial differentiation and age: A study of 1,010 diffuse gliomas, Acta Neuropathol., 118, 469, 10.1007/s00401-009-0561-9

Ye, 2012, The mechanisms of IDH mutations in tumorigenesis, Cell Res., 22, 1102, 10.1038/cr.2012.51

Xu, 2011, Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases, Cancer Cell, 19, 17, 10.1016/j.ccr.2010.12.014

Chowdhury, 2011, The oncometabolite 2-hydroxyglutarate inhibits histone lysine demethylases, EMBO Rep., 12, 463, 10.1038/embor.2011.43

Turcan, 2012, IDH1 mutation is sufficient to establish the glioma hypermethylator phenotype, Nature, 483, 479, 10.1038/nature10866

Lu, 2012, IDH mutation impairs histone demethylation and results in a block to cell differentiation, Nature, 483, 474, 10.1038/nature10860

Noorani, I. (2019). Genetically engineered mouse models of gliomas: Technological developments for translational discoveries. Cancers, 11.

Philip, 2018, Mutant IDH1 Promotes Glioma Formation In Vivo, Cell Rep., 23, 1553, 10.1016/j.celrep.2018.03.133

Bettegowda, 2011, Mutations in CIC and FUBP1 Contribute to Human Oligodendroglioma, Science, 333, 1453, 10.1126/science.1210557

Yip, 2012, Concurrent CIC mutations, IDH mutations, and 1p/19q loss distinguish oligodendrogliomas from other cancers, J. Pathol., 226, 7, 10.1002/path.2995

The Cancer Genome Atlas Research Network (2015). Comprehensive, Integrative Genomic Analysis of Diffuse Lower-Grade Gliomas. N. Engl. J. Med., 372, 2481–2498.

Suzuki, 2015, Mutational landscape and clonal architecture in grade II and III gliomas, Nat. Genet., 47, 458, 10.1038/ng.3273

Metellus, 2010, Absence of IDH mutation identifies a novel radiologic and molecular subtype of WHO grade II gliomas with dismal prognosis, Acta Neuropathol., 120, 719, 10.1007/s00401-010-0777-8

Noorani, 2020, PiggyBac mutagenesis and exome sequencing identify genetic driver landscapes and potential therapeutic targets of EGFR-mutant gliomas, Genome Biol., 21, 181, 10.1186/s13059-020-02092-2

Nobusawa, 2009, IDH1 mutations as molecular signature and predictive factor of sec-ondary glioblastomas, Clin. Cancer Res., 15, 6002, 10.1158/1078-0432.CCR-09-0715

Ohgaki, 2004, Genetic pathways to glioblastoma: A population-based study, Cancer Res., 64, 6892, 10.1158/0008-5472.CAN-04-1337

Bender, 2013, Reduced H3K27me3 and DNA Hypomethylation Are Major Drivers of Gene Expression in K27M Mutant Pediatric High-Grade Gliomas, Cancer Cell, 24, 660, 10.1016/j.ccr.2013.10.006

Johnson, 2014, Mutational Analysis Reveals the Origin and Therapy-Driven Evolution of Recurrent Glioma, Science, 343, 189, 10.1126/science.1239947

Watanabe, 2009, IDH1 mutations are early events in the development of astrocytomas and oligodendrogliomas, Am. J. Pathol., 174, 1149, 10.2353/ajpath.2009.080958

Lai, 2011, Evidence for sequenced molecular evolution of IDH1 mutant glioblastoma from a distinct cell of origin, J. Clin. Oncol., 29, 4482, 10.1200/JCO.2010.33.8715

Rohle, 2013, An inhibitor of mutant IDH1 delays growth and promotes differentiation of glioma cells, Science, 340, 626, 10.1126/science.1236062

Wang, 2013, Targeted Inhibition of Mutant IDH2 in Leukemia Cells Induces Cellular Differentiation, Science, 340, 622, 10.1126/science.1234769

Bartholdy, 2015, New IDH1 mutant inhibitors for treatment of acute myeloid leukemia, Nat. Chem. Biol., 11, 878, 10.1038/nchembio.1930

Grassian, 2014, IDH1 mutations alter citric acid cycle metabolism and increase dependence on oxidative mitochondrial metabolism, Cancer Res., 74, 3317, 10.1158/0008-5472.CAN-14-0772-T

Horton, 2016, Char-acterization of a Linked Jumonji Domain of the KDM5/JARID1 Family of Histone H3 Lysine 4 Demethylases, J. Biol. Chem., 291, 2631, 10.1074/jbc.M115.698449

Chesnelong, 2014, Lactate dehydrogenase A silencing in IDH mutant gliomas, Neuro-Oncology, 16, 686, 10.1093/neuonc/not243

Kayabolen, A., Sahin, G.N., Seker-Polat, F., Cingoz, A., Isik, B., Acar, S., Wakimoto, H., Cahill, D.P., Solaroglu, I., and Cribbs, A. (2020). Combined inhibition of KDM6A/B and HDACs exacerbates integrated stress response and mediates therapeutic effects in IDH1-mutant glioma. bioRxiv.

Kopinja, 2017, A Brain Pen-etrant Mutant IDH1 Inhibitor Provides in Vivo Survival Benefit, Sci. Rep., 7, 13853, 10.1038/s41598-017-14065-w

Pusch, 2017, Pan-mutant IDH1 inhibitor BAY 1436032 for effective treatment of IDH1 mutant astrocytoma in vivo, Acta Neuropathol., 133, 629, 10.1007/s00401-017-1677-y

Turcan, 2013, Efficient induction of differentiation and growth inhibition in IDH1 mutant glioma cells by the DNMT Inhibitor Decita-bine, Oncotarget, 4, 1729, 10.18632/oncotarget.1412

Molenaar, 2015, Radioprotection of IDH1-mutated cancer cells by the IDH1-mutant inhibitor AGI-5198, Cancer Res., 75, 4790, 10.1158/0008-5472.CAN-14-3603

Sulkowski, 2017, 2-Hydroxyglutarate produced by neomorphic IDH mutations suppresses homologous recombination and induces PARP inhibitor sensitivity, Sci. Transl. Med., 9, eaal2463, 10.1126/scitranslmed.aal2463

Kim, 2017, Avelumab: First Global Approval, Drugs, 77, 929, 10.1007/s40265-017-0749-6

Norsworthy, 2019, FDA Approval Summary: Ivosidenib for Relapsed or Refractory Acute Myeloid Leukemia with an Isocitrate Dehydrogenase-1 Mutation, Clin. Cancer Res., 25, 3205, 10.1158/1078-0432.CCR-18-3749

Andronesi, O.C., Arrillaga-Romany, I.C., Ly, K.I., Bogner, W., Ratai, E.M., Reitz, K., Iafrate, A.J., Dietrich, J., Gerstner, E.R., and Chi, A.S. (2018). Pharmacodynamics of mutant-IDH1 inhibitors in glioma patients probed by in vivo 3D MRS imaging of 2-hydroxyglutarate. Nat. Commun., 9.

Tateishi, 2015, Extreme Vulnerability of IDH1 Mutant Cancers to NAD+ Depletion, Cancer Cell, 28, 773, 10.1016/j.ccell.2015.11.006

DiNardo, 2018, Durable remissions with ivosidenib in IDH1-mutated relapsed or refractory AML, N. Engl. J. Med., 378, 2386, 10.1056/NEJMoa1716984

Turcan, 2018, Mutant-IDH1-dependent chromatin state reprogramming, reversibility, and persistence, Nat. Genet., 50, 62, 10.1038/s41588-017-0001-z

Johannessen, 2016, Rapid Conversion of Mutant IDH1 from Driver to Passenger in a Model of Human Gliomagenesis, Mol. Cancer Res., 14, 976, 10.1158/1541-7786.MCR-16-0141

Kats, 2014, Proto-oncogenic role of mutant IDH2 in leukemia initiation and maintenance, Cell Stem Cell, 14, 329, 10.1016/j.stem.2013.12.016

Mellinghoff, 2020, Ivosidenib in Isocitrate Dehydrogenase 1-Mutated Advanced Glioma, J. Clin. Oncol., 38, 3398, 10.1200/JCO.19.03327

Natsume, 2019, Phase I study of a brain penetrant mutant IDH1 inhibitor DS-1001b in patients with recurrent or progressive IDH1 mutant gliomas, J. Clin. Oncol., 37, 2004, 10.1200/JCO.2019.37.15_suppl.2004

DiNardo, 2016, A Phase I Study of IDH305 in Patients with Advanced Malignancies Including Relapsed/Refractory AML and MDS That Harbor IDH1R132 Mutations, Blood, 128, 1073, 10.1182/blood.V128.22.1073.1073

Colman, 2020, A phase Ib/II study of olutasidenib in patients with relapsed/refractory IDH1 mutant gliomas: Safety and efficacy as single agent and in combination with azacitidine, J. Clin. Oncol., 38, 2505, 10.1200/JCO.2020.38.15_suppl.2505

Erridge, 2019, Second interim and first molecular analysis of the EORTC randomized phase III intergroup CAT-NON trial on concurrent and adjuvant temozolomide in anaplastic glioma without 1p/19q codeletion, J. Clin. Oncol., 37, 2000, 10.1200/JCO.2019.37.15_suppl.2000

Warburg, 1956, On the origin of cancer cells, Science, 123, 309, 10.1126/science.123.3191.309

Zecchini, 2017, Metabolic synthetic lethality in cancer therapy. Biochim. Biophys, Acta Bioenerg., 1858, 723, 10.1016/j.bbabio.2016.12.003

Jo, 2001, Control of Mitochondrial Redox Balance and Cellular Defense against Oxidative Damage by Mitochondrial NADP+-dependent Isocitrate Dehydrogenase, J. Biol. Chem., 276, 16168, 10.1074/jbc.M010120200

Koh, 2004, Cytosolic NADP+-dependent isocitrate dehydrogenase plays a key role in lipid metabolism, J. Biol. Chem., 279, 39968, 10.1074/jbc.M402260200

Badur, 2018, Onco-genic R132 IDH1 Mutations Limit NADPH for De Novo Lipogenesis through (D)2-Hydroxyglutarate Production in Fibro-sarcoma Sells, Cell Rep., 25, 1018, 10.1016/j.celrep.2018.09.074

Lita, A., Pliss, A., Kuzmin, A., Yamasaki, T., Zhang, L., Dowdy, T., Burks, C., de Val, N., Celiku, O., and Ruiz-Rodado, V. (2020). IDH1 Mutations Induce Organelle Defects Via Dysregulated Phospholipids. bioRxiv.

Dowdy, T., Zhang, L., Celiku, O., Movva, S., Lita, A., Ruiz-Rodado, V., Gilbert, M.R., and Larion, M. (2020). Sphingolipid pathway as a source of vulnerability in IDH1mut glioma. Cancers, 12.

Newsholme, 2003, Glutamine and glutamate?their central role in cell metabolism and function, Cell Biochem. Funct., 21, 1, 10.1002/cbf.1003

Altman, 2016, From Krebs to clinic: Glutamine metabolism to cancer therapy, Nat. Rev. Cancer, 16, 619, 10.1038/nrc.2016.71

Fack, 2017, Altered metabolic landscape in IDH-mutant gliomas affects phospholipid, energy, and oxidative stress pathways, EMBO Mol. Med., 9, 1681, 10.15252/emmm.201707729

Shah, 2017, In Vivo Imaging of Glutamine Metabolism to the Oncometabolite 2-Hydroxyglutarate in IDH1/2 Mu-tant Tumors, Cell Metab., 26, 830, 10.1016/j.cmet.2017.10.001

Seltzer, 2010, Inhibition of glutaminase preferentially slows growth of glioma cells with mutant IDH1, Cancer Res., 70, 8981, 10.1158/0008-5472.CAN-10-1666

Chen, 2014, Hominoid-specific enzyme GLUD2 promotes growth of IDH1 R132H glioma, Proc. Natl. Acad. Sci. USA, 111, 14217, 10.1073/pnas.1409653111

Burki, 2004, Birth and adaptive evolution of a hominoid gene that supports high neurotransmitter flux, Nat. Genet., 36, 1061, 10.1038/ng1431

Shashidharan, 2014, The discovery of human of GLUD2 glutamate dehydrogenase and its implications for cell function in health and disease, Neurochem. Res., 39, 460, 10.1007/s11064-013-1227-5

McBrayer, 2018, Transaminase Inhibition by 2-Hydroxyglutarate Impairs Glutamate Biosynthesis and Redox Homeostasis in Glioma, Cell, 175, 101, 10.1016/j.cell.2018.08.038

Tateishi, 2017, The Alkylating Chemotherapeutic Temozolomide Induces Metabolic Stress in IDH1 -Mutant Cancers and Poten-tiates NAD + Depletion–Mediated Cytotoxicity, Cancer Res., 77, 4102, 10.1158/0008-5472.CAN-16-2263

Miller, 2020, Sirtuin activation targets IDH-mutant tumors, Neuro-Oncology, 23, 53, 10.1093/neuonc/noaa180

Nagashima, 2020, Poly(ADP-ribose) Glycohydrolase Inhibition Sequesters NAD+ to Potentiate the Metabolic Lethality of Alkyl-ating Chemotherapy in IDH-Mutant Tumor Cells, Cancer Discov., 10, 1672, 10.1158/2159-8290.CD-20-0226

Lu, 2017, Chemosensitivity of IDH1-mutated gliomas due to an impairment in PARP1-mediated DNA repair, Cancer Res., 77, 1709, 10.1158/0008-5472.CAN-16-2773

Chan, 2015, Isocitrate dehydrogenase 1 and 2 mutations induce BCL-2 dependence in acute myeloid leukemia, Nat. Med., 21, 178, 10.1038/nm.3788

Gelman, 2018, Consumption of NADPH for 2-HG Synthesis Increases Pentose Phosphate Pathway Flux and Sensitizes Cells to Oxidative Stress, Cell Rep., 22, 512, 10.1016/j.celrep.2017.12.050

Holmgren, 2010, Thioredoxin and thioredoxin reductase: Current research with special reference to human disease, Biochem. Biophys. Res. Commun., 396, 120, 10.1016/j.bbrc.2010.03.083

Fu, 2015, 2-hydroxyglutarate inhibits ATP synthase and mTOR Signaling, Cell Metab., 22, 508, 10.1016/j.cmet.2015.06.009

Ishida, 2017, Induction of synthetic lethality in IDH1-mutated gliomas through inhibition of Bcl-xL, Nat. Commun., 8, 1067, 10.1038/s41467-017-00984-9

Carbonneau, M., Gagne, L.M., Lalonde, M.E., Germain, M.A., Motorina, A., Guiot, M.C., Secco, B., Vincent, E.E., Tumber, A., and Hulea, L. (2016). The oncometabolite 2-hydroxyglutarate activates the mTOR signalling pathway. Nat. Commun., 7.

Batsios, 2019, PI3K/mTOR inhibition of IDH1 mutant glioma leads to reduced 2HG production that is associated with increased survival, Sci. Rep., 9, 10521, 10.1038/s41598-019-47021-x

Wakimoto, 2014, Targetable Signaling Pathway Mutations Are Associated with Malignant Phenotype in IDH-Mutant Gliomas, Clin. Cancer Res., 20, 2898, 10.1158/1078-0432.CCR-13-3052

Fan, 2006, A dual PI3 ki-nase/mTOR inhibitor reveals emergent efficacy in glioma, Cancer Cell, 9, 341, 10.1016/j.ccr.2006.03.029

Puli, 2010, Effect of combination treatment of rapamycin and isoflavones on mtor pathway in human glioblastoma (U87) cells, Neurochem. Res., 35, 986, 10.1007/s11064-010-0142-2

Sasaki, 2012, D-2-hydroxyglutarate produced by mutant IDH1 perturbs collagen maturation and basement membrane function, Genes Dev., 26, 2038, 10.1101/gad.198200.112

Viswanath, 2018, 2-Hydroxyglutarate-Mediated Autophagy of the Endoplasmic Reticulum Leads To an Unusual Down-regulation of Phospholipid Biosynthesis in Mutant IDH1 Gliomas, Cancer Res., 78, 2290, 10.1158/0008-5472.CAN-17-2926

Zhang, 2019, Mutant IDH sensitizes gliomas to endoplasmic reticulum stress and triggers apoptosis via miR-183-mediated inhibition of semaphorin 3E, Cancer Res., 79, 4994, 10.1158/0008-5472.CAN-19-0054

Ou, X., Liu, Y., Lei, X., Li, P., Mi, D., Ren, L., Guo, L., Guo, R., Chen, T., and Hu, J. (2020). Characterization of spike glycoprotein of SARS-CoV-2 on virus entry and its immune cross-reactivity with SARS-CoV. Nat. Commun., 11.

Yalaza, 2017, R132H mutation in IDH1 gene is associated with increased tumor HIF1-alpha and serum VEGF levels in primary glioblastoma multiforme, Ann. Clin. Lab. Sci., 47, 362

Jensen, 2006, Hypoxia in the tumorigenesis of gliomas and as a potential target for therapeutic measures, Neurosurg. Focus, 20, E24, 10.3171/foc.2006.20.4.16

Gillespie, 2007, Silencing of hypoxia inducible factor-1α by RNA interference attenuates human glioma cell growth in vivo, Clin. Cancer Res., 13, 2441, 10.1158/1078-0432.CCR-06-2692

Fujiwara, 2007, Si-lencing hypoxia-inducible factor-1α inhibits cell migration and invasion under hypoxic environment in malignant gliomas, Int. J. Oncol., 30, 793

Tang, 2016, Downregulation of HIF-1a sensitizes U251 glioma cells to the temozolomide (TMZ) treatment, Exp. Cell Res., 343, 148, 10.1016/j.yexcr.2016.04.011

Kessler, J., Hahnel, A., Wichmann, H., Rot, S., Kappler, M., Bache, M., and Vordermark, D. (2010). HIF-1α inhibition by siRNA or chetomin in human malignant glioma cells: Effects on hypoxic radioresistance and monitoring via CA9 expression. BMC Cancer, 10.

Koivunen, 2012, Transformation by the (R)-enantiomer of 2-hydroxyglutarate linked to EGLN activation, Nature, 483, 484, 10.1038/nature10898

Hartmann, 2010, Patients with IDH1 wild type anaplastic astrocytomas exhibit worse prognosis than IDH1-mutated glioblas-tomas, and IDH1 mutation status accounts for the unfavorable prognostic effect of higher age: Implications for classification of gliomas, Acta Neuropathol., 120, 707, 10.1007/s00401-010-0781-z

Intlekofer, 2015, Hypoxia Induces Production of L-2-Hydroxyglutarate, Cell Metab., 22, 304, 10.1016/j.cmet.2015.06.023

Houillier, 2010, IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade glio-mas, Neurology, 75, 1560, 10.1212/WNL.0b013e3181f96282

SongTao, 2012, IDH mutations predict longer survival and response to temozolomide in secondary glioblastoma, Cancer Sci., 103, 269, 10.1111/j.1349-7006.2011.02134.x

Dubbink, 2010, IDH1 and IDH2 mutations are prognostic but not predictive for outcome in anaplastic oli-godendroglial tumors: A report of the European Organization for Research and Treatment of Cancer Brain Tumor Group, Clin. Cancer Res., 16, 1597, 10.1158/1078-0432.CCR-09-2902

Li, 2013, Overex-pression of isocitrate dehydrogenase 1/2 (IDH1/2) mutant protein renders glioma cells more sensitive to radiation, Neuro-Oncology, 15, 57, 10.1093/neuonc/nos261

Tran, 2014, Increased sensitivity to radiochemotherapy in IDH1 mutant glioblastoma as demonstrated by serial quantita-tive MR volumetry, Neuro-Oncology, 16, 414, 10.1093/neuonc/not198

Buckner, 2016, Radiation plus Procarbazine, CCNU, and Vincristine in Low-Grade Glioma, N. Engl. J. Med., 374, 1344, 10.1056/NEJMoa1500925

Sanson, 2009, Isocitrate dehydrogenase 1 codon 132 mutation is an important prognostic biomarker in gliomas, J. Clin. Oncol., 27, 4150, 10.1200/JCO.2009.21.9832

Claus, 2015, Survival and low-grade glioma: The emergence of genetic information, Neurosurg. Focus., 38, E6, 10.3171/2014.10.FOCUS12367

Wang, 2020, Target-ing therapeutic vulnerabilities with PARP inhibition and radiation in IDH-mutant gliomas and cholangiocarcinomas, Sci. Adv., 6, eaaz3221, 10.1126/sciadv.aaz3221

Platten, 2021, A vac-cine targeting mutant IDH1 in newly diagnosed glioma, Nature, 592, 463, 10.1038/s41586-021-03363-z

Hodges, 2017, Muta-tional burden, immune checkpoint expression, and mismatch repair in glioma: Implications for immune checkpoint im-munotherapy, Neuro-Oncology, 19, 1047, 10.1093/neuonc/nox026

Qazi, 2017, Intratumoral heterogeneity: Pathways to treatment resistance and relapse in human glioblastoma, Ann. Oncol., 28, 1448, 10.1093/annonc/mdx169

Schumacher, 2014, A vac-cine targeting mutant IDH1 induces antitumour immunity, Nature, 512, 324, 10.1038/nature13387

Kohanbash, 2017, Isocitrate dehydrogenase mutations suppress STAT1 and CD8+ T cell accumulation in gliomas, J. Clin. Invest., 127, 1425, 10.1172/JCI90644

Berghoff, 2017, Correlation of immune phenotype with IDH mutation in diffuse glioma, Neuro-Oncology, 19, 1460, 10.1093/neuonc/nox054

Amankulor, 2017, Mutant IDH1 regulates the tumor-associated immune system in gliomas, Genes Dev., 31, 774, 10.1101/gad.294991.116

Bunse, 2018, Suppression of antitumor T cell immunity by the oncometabolite (R)-2-hydroxyglutarate, Nat. Med., 24, 1192, 10.1038/s41591-018-0095-6

Kadiyala, P., Carney, S.V., Gauss, J.C., Garcia-Fabiani, M.B., Haase, S., Alghamri, M.S., Núñez, F.J., Liu, Y., Yu, M., and Taher, A.W. (2020). Inhibition of 2-Hydroxyglutarate Elicits Metabolic-reprograming and Mutant IDH1 Glioma Immunity in Mice. J. Clin. Invest.

Pellegatta, 2015, Effective immuno-targeting of the IDH1 mutation R132H in a murine model of intracranial glioma, Acta Neuropathol. Commun., 3, 4, 10.1186/s40478-014-0180-0

Platten, 2018, ATIM-33. NOA-16: A first-in-man multicenter phase i clinical trial of the german neurooncology working group evaluating a mutation-specific peptide vaccine targeting idh1r132h in patients with newly diagnosed malignant as-trocytomas, Neuro-Oncology, 20, vi8, 10.1093/neuonc/noy148.028

Sampson, 2014, EGFRvIII mCAR-modified T-cell therapy cures mice with established intracerebral glioma and generates host immunity against tumor-antigen loss, Clin. Cancer Res., 20, 972, 10.1158/1078-0432.CCR-13-0709

O’Rourke, D.M., Nasrallah, M.P., Desai, A., Melenhorst, J.J., Mansfield, K., Morrissette, J.J.D., Martinez-Lage, M., Brem, S., Maloney, E., and Shen, A. (2017). A single dose of peripherally infused EGFRvIII-directed CAR T cells mediates antigen loss and induces adaptive resistance in patients with recurrent glioblastoma. Sci. Transl. Med., 9.

Senhaji, N., Louati, S., Chbani, L., el Fatemi, H., Hammas, N., Mikou, K., Maaroufi, M., Benzagmout, M., Boujraf, S., and el Bardai, S. (2017). EGFR Amplification and IDH Mutations in Glioblastoma Patients of the Northeast of Morocco. Biomed Res. Int.

Taher, 2020, EGFRvIII expression and isocitrate dehydrogenase mutations in patients with glioma, Oncol. Lett., 20, 384, 10.3892/ol.2020.12247

Flavahan, 2016, Insulator dysfunction and oncogene activation in IDH mutant gliomas, Nature, 529, 110, 10.1038/nature16490

Xiao, 2020, Chimeric antigen recep-tor-modified T-cell therapy for platelet-derived growth factor receptor α-positive rhabdomyosarcoma, Cancer, 126, 2093, 10.1002/cncr.32764

Borodovsky, A., Salmasi, V., Turcan, S., Fabius, A.W.M., Baia, G., Eberhart, C.G., Weingart, J.D., Gallia, G.L., Baylin, S.B., and Chan, T.A. (2013). 5-azacytidine reduces methylation, promotes differentiation and induces tumor regression in a pa-tient-derived IDH1 mutant glioma xenograft. Oncotarget, 4.

Yamashita, 2019, Demethylation and epigenetic modifica-tion with 5-azacytidine reduces IDH1 mutant glioma growth in combination with temozolomide, Neuro-Oncology, 21, 189, 10.1093/neuonc/noy146

Oganesian, 2013, Preclinical data in cynomolgus (cyn) monkeys of ASTX727, a novel oral hypomethylating agent (HMA) composed of low-dose oral decitabine combined with a novel cytidine deami-nase inhibitor (CDAi) E7727, Blood, 122, 2526, 10.1182/blood.V122.21.2526.2526

Griffiths, 2020, Oral cedazuridine/decitabine for MDS and CMML: A phase 2 pharmacokinetic/pharmacodynamic random-ized crossover study, Blood, 136, 674, 10.1182/blood.2019004143

Bai, 2016, Integrated genomic characterization of IDH1-mutant glioma malignant progression, Nat. Genet., 48, 59, 10.1038/ng.3457