IDH2 Mutations in Patients with d -2-Hydroxyglutaric Aciduria

An Integrated Genomic Analysis of Human Glioblastoma Multiforme

Parsons, D. W., Jones, S., Zhang, X., Lin, J. C.-H., Leary, R. J., Angenendt, P., Mankoo, P., Carter, H., Siu, I.-M., Gallia, G. L., et al. (2008) · Science · doi:10.1126/science.1164382 · Cited by 5,857

IDH1 and IDH2 Mutations in Gliomas

Yan, H., Parsons, D. W., Jin, G., McLendon, R., Rasheed, B. A., Yuan, W., Kos, I., Batinic-Haberle, I., Jones, S., Riggins, G. J., et al. (2009) · New England Journal of Medicine · doi:10.1056/nejmoa0808710 · Cited by 5,847

Cancer-associated IDH1 mutations produce 2-hydroxyglutarate

Dang, L., White, D. W., Gross, S., Bennett, B. D., Bittinger, M. A., Driggers, E. M., Fantin, V. R., Jang, H. G., Jin, S., Keenan, M. C., et al. (2009) · Nature · doi:10.1038/nature08617 · Cited by 4,154

The Common Feature of Leukemia-Associated IDH1 and IDH2 Mutations Is a Neomorphic Enzyme Activity Converting α-Ketoglutarate to 2-Hydroxyglutarate

Ward, P. S., Patel, J., Wise, D. R., Abdel-Wahab, O., Bennett, B. D., Coller, H. A., Cross, J. R., Fantin, V. R., Hedvat, C. V., Perl, A. E., et al. (2010) · Cancer Cell · doi:10.1016/j.ccr.2010.01.020 · Cited by 2,020

Mutations in the d-2-Hydroxyglutarate Dehydrogenase Gene Cause d-2-Hydroxyglutaric Aciduria

Struys, E. A., Salomons, G. S., Achouri, Y., Van Schaftingen, E., Grosso, S., Craigen, W. J., Verhoeven, N. M., & Jakobs, C. (2005) · The American Journal of Human Genetics · doi:10.1086/427890 · Cited by 172

Hydroxyglutaric aciduria and malignant brain tumor: a case report and literature review

Aghili, M., Zahedi, F., & Rafiee, E. (2009) · Journal of Neuro-Oncology · doi:10.1007/s11060-008-9706-2 · Cited by 159

An overview of L-2-hydroxyglutarate dehydrogenase gene (L2HGDH) variants: a genotype-phenotype study

Steenweg, M. E., Jakobs, C., Errami, A., van Dooren, S. J. M., Bartolomé, M. T. A., Aerssens, P., Augoustides‐Savvopoulou, P., Barić, I., Baumann, M., Bonafé, L., et al. (2010) · Human Mutation · doi:10.1002/humu.21197 · Cited by 128

Evidence for genetic heterogeneity in D-2-hydroxyglutaric aciduria

Kranendijk, M., Struys, E. A., Gibson, K. M., Wickenhagen, W. V., Abdenur, J. E., Buechner, J., Christensen, E., de Kremer, R. D., Errami, A., Gissen, P., et al. (2009) · Human Mutation · doi:10.1002/humu.21186 · Cited by 74

Investigations by mass isotopomer analysis of the formation of D ‐2‐hydroxyglutarate by cultured lymphoblasts from two patients with D ‐2‐hydroxyglutaric aciduria

Struys, E. A., Verhoeven‐Duif, N. M., Brunengraber, H., & Jakobs, C. (2003) · FEBS Letters · doi:10.1016/s0014-5793(03)01459-5 · Cited by 40

Metabolic Reprogramming: A Cancer Hallmark Even Warburg Did Not Anticipate

Ward, P. S., & Thompson, C. B. (2012) · Cancer Cell · doi:10.1016/j.ccr.2012.02.014 · Cited by 3,233

The Control of the Metabolic Switch in Cancers by Oncogenes and Tumor Suppressor Genes

Levine, A. J., & Puzio-Kuter, A. M. (2010) · Science · doi:10.1126/science.1193494 · Cited by 1,243

IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours

Amary, M. F., Bacsi, K., Maggiani, F., Damato, S., Halai, D., Berisha, F., Pollock, R., O'Donnell, P., Grigoriadis, A., Diss, T., et al. (2011) · The Journal of Pathology · doi:10.1002/path.2913 · Cited by 1,023

SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways

Park, J., Chen, Y., Tishkoff, D. X., Peng, C., Tan, M., Dai, L., Xie, Z., Zhang, Y., Zwaans, B. M. M., Skinner, M. E., et al. (2013) · Molecular Cell · doi:10.1016/j.molcel.2013.06.001 · Cited by 1,000

Cellular Metabolism and Disease: What Do Metabolic Outliers Teach Us?

DeBerardinis, R. J., & Thompson, C. B. (2012) · Cell · doi:10.1016/j.cell.2012.02.032 · Cited by 858

Emerging insights into the molecular and cellular basis of glioblastomaPreprint

Dunn, G. P., Rinne, M. L., Wykosky, J., Genovese, G., Quayle, S. N., Dunn, I. F., Agarwalla, P. K., Chheda, M. G., Campos, B., Wang, A., et al. (2012) · Genes & Development · doi:10.1101/gad.187922.112 · Cited by 573

Malignant Glioma: Lessons from Genomics, Mouse Models, and Stem Cells

Chen, J., McKay, R. M., & Parada, L. F. (2012) · Cell · doi:10.1016/j.cell.2012.03.009 · Cited by 549

IDH mutations in cancer and progress toward development of targeted therapeutics

Dang, L., Yen, K., & Attar, E. C. (2016) · Annals of Oncology · doi:10.1093/annonc/mdw013 · Cited by 509

IDH1andIDH2Mutations in Tumorigenesis: Mechanistic Insights and Clinical Perspectives

Yang, H., Ye, D., Guan, K., & Xiong, Y. (2012) · Clinical Cancer Research · doi:10.1158/1078-0432.ccr-12-1773 · Cited by 477

Oncogenic Isocitrate Dehydrogenase Mutations: Mechanisms, Models, and Clinical Opportunities

Cairns, R. A., & Mak, T. W. (2013) · Cancer Discovery · doi:10.1158/2159-8290.cd-13-0083 · Cited by 452

Oncometabolites: linking altered metabolism with cancer

Yang, M. H., Soga, T., & Pollard, P. J. (2013) · Journal of Clinical Investigation · doi:10.1172/jci67228 · Cited by 441

Coupling Krebs cycle metabolites to signalling in immunity and cancer

Ryan, D. G., Murphy, M. P., Frezza, C., Prag, H. A., Chouchani, E. T., O’Neill, L. A., & Mills, E. L. (2018) · Nature Metabolism · doi:10.1038/s42255-018-0014-7 · Cited by 398

Dysregulation of glucose transport, glycolysis, TCA cycle and glutaminolysis by oncogenes and tumor suppressors in cancer cells

Chen, J., & Russo, J. (2012) · Biochimica et Biophysica Acta (BBA) - Reviews on Cancer · doi:10.1016/j.bbcan.2012.06.004 · Cited by 340

Metabolic signatures of cancer cells and stem cells

Intlekofer, A. M., & Finley, L. W. S. (2019) · Nature Metabolism · doi:10.1038/s42255-019-0032-0 · Cited by 322

Progress in understanding 2‐hydroxyglutaric acidurias

Kranendijk, M., Struys, E. A., Salomons, G. S., Van der Knaap, M. S., & Jakobs, C. (2012) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-012-9462-5 · Cited by 292

Metabolite damage and its repair or pre-emption

Linster, C. L., SCHAFTINGEN, E. V., & Hanson, A. D. (2013) · Nature Chemical Biology · doi:10.1038/nchembio.1141 · Cited by 273

Revisiting the TCA cycle: signaling to tumor formation

Raimundo, N., Baysal, B. E., & Shadel, G. S. (2011) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2011.06.001 · Cited by 281

Signaling in Control of Cell Growth and MetabolismPreprint

Ward, P. S., & Thompson, C. B. (2012) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a006783 · Cited by 289

Metabolism, Activity, and Targeting of D- and L-2-Hydroxyglutarates

Ye, D., Guan, K.-L., & Xiong, Y. (2018) · Trends in Cancer · doi:10.1016/j.trecan.2017.12.005 · Cited by 242

2-Hydroxyglutarate Inhibits ATP Synthase and mTOR Signaling

Fu, X., Chin, R., Vergnes, L., Hwang, H., Deng, G., Xing, Y., Pai, M. Y., Li, S., Ta, L. H., Fazlollahi, F., et al. (2015) · Cell Metabolism · doi:10.1016/j.cmet.2015.06.009 · Cited by 230

The cancer driver genes IDH1/2, JARID1C/ KDM5C, and UTX/ KDM6A: crosstalk between histone demethylation and hypoxic reprogramming in cancer metabolism

Chang, S., Yim, S., & Park, H. (2019) · Experimental & Molecular Medicine · doi:10.1038/s12276-019-0230-6 · Cited by 228

IDH Mutation in Glioma

Turkalp, Z. T., Karamchandani, J., & Das, S. (2014) · JAMA Neurology · doi:10.1001/jamaneurol.2014.1205 · Cited by 222

IDH1 and IDH2 mutations as novel therapeutic targets: current perspectives

de Botton, S., Mondesir, J., Willekens, C., & Touat, M. (2016) · Journal of Blood Medicine · doi:10.2147/jbm.s70716 · Cited by 220

Oncometabolites in renal cancer

Yong, C., Stewart, G. D., & Frezza, C. (2019) · Nature Reviews Nephrology · doi:10.1038/s41581-019-0210-z · Cited by 216

Exploring the molecular interface between hypoxia-inducible factor signalling and mitochondria

Thomas, L. W., & Ashcroft, M. A. (2019) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-019-03039-y · Cited by 219

Isocitrate Dehydrogenase Mutation and ( R )-2-Hydroxyglutarate: From Basic Discovery to Therapeutics Development

Dang, L., & Su, S.-S. M. (2017) · Annual Review of Biochemistry · doi:10.1146/annurev-biochem-061516-044732 · Cited by 204

Identification of additional IDH mutations associated with oncometabolite R(−)-2-hydroxyglutarate production

Ward, P. S., Cross, J. R., Lü, C., Weigert, O., Abdel-Wahab, O. I., Levine, R. L., Weinstock, D. M., Sharp, K. A., & Thompson, C. B. (2011) · Oncogene · doi:10.1038/onc.2011.416 · Cited by 197

Targeting Metabolic Remodeling in Glioblastoma Multiforme

Wolf, A., Agnihotri, S., & Guha, A. (2010) · Oncotarget · doi:10.18632/oncotarget.190 · Cited by 194

The driver and passenger effects of isocitrate dehydrogenase 1 and 2 mutations in oncogenesis and survival prolongation

Molenaar, R. J., Radivoyevitch, T., Maciejewski, J. P., Noorden, C. J. F. V., & Bleeker, F. E. (2014) · Biochimica et Biophysica Acta (BBA) - Reviews on Cancer · doi:10.1016/j.bbcan.2014.05.004 · Cited by 186

The Potential for Isocitrate Dehydrogenase Mutations to Produce 2-Hydroxyglutarate Depends on Allele Specificity and Subcellular Compartmentalization

Ward, P. S., Lü, C., Cross, J. R., Abdel-Wahab, O. I., Levine, R. L., Schwartz, G. K., & Thompson, C. B. (2012) · Journal of Biological Chemistry · doi:10.1074/jbc.m112.435495 · Cited by 188

Cancer-associated mutation and beyond: The emerging biology of isocitrate dehydrogenases in human disease

Tommasini-Ghelfi, S., Murnan, K., Kouri, F. M., Mahajan, A., May, J., & Stegh, A. H. (2019) · Science Advances · doi:10.1126/sciadv.aaw4543 · Cited by 166

What do we know about IDH1/2 mutations so far, and how do we use it?

Horbinski, C. (2013) · Acta Neuropathologica · doi:10.1007/s00401-013-1106-9 · Cited by 169

Recommendations for Cancer Surveillance in Individuals with RASopathies and Other Rare Genetic Conditions with Increased Cancer Risk

Villani, A., Greer, M. C., Kalish, J. M., Nakagawara, A., Nathanson, K. L., Pajtler, K. W., Pfister, S. M., Walsh, M. F., Wasserman, J. D., Zelley, K., et al. (2017) · Clinical Cancer Research · doi:10.1158/1078-0432.ccr-17-0631 · Cited by 167

Metabolic consequences of oncogenic IDH mutations

Parker, S. J., & Metallo, C. M. (2015) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2015.05.003 · Cited by 162

The Roles of 2-Hydroxyglutarate

Du, X., & Hu, H. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2021.651317 · Cited by 160

Targeting cancer metabolism

Teicher, B. A., Linehan, W. M., & Helman, L. J. (2012) · Clinical Cancer Research · doi:10.1158/1078-0432.ccr-12-2587 · Cited by 152

Isocitrate Dehydrogenase Mutations in Glioma: From Basic Discovery to Therapeutics Development

Huang, J., Yu, J., Tu, L., Huang, N., Li, H., & Luo, Y. (2019) · Frontiers in Oncology · doi:10.3389/fonc.2019.00506 · Cited by 147

Oncometabolite d -2-hydroxyglutarate impairs α-ketoglutarate dehydrogenase and contractile function in rodent heart

Karlstaedt, A., Zhang, X., Vitrac, H., Harmancey, R., Vasquez, H., Wang, J. H., Goodell, M. A., & Taegtmeyer, H. (2016) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1601650113 · Cited by 144

Oncogenic Activities of IDH1/2 Mutations: From Epigenetics to Cellular Signaling

Gagné, L. M., Boulay, K., Topisirović, I., Huot, M., & Mallette, F. A. (2017) · Trends in Cell Biology · doi:10.1016/j.tcb.2017.06.002 · Cited by 143

IDH1 R132H decreases proliferation of glioma cell lines in vitro and in vivo

Bralten, L. B. C., Kloosterhof, N. K., Balvers, R. K., Sacchetti, A., Lapre, L., Lamfers, M. L. M., Leenstra, S., de Jonge, H. R., Kros, J. M., Jansen, E. E. W., et al. (2011) · Annals of Neurology · doi:10.1002/ana.22390 · Cited by 148

Deficiency in SLC25A1, Encoding the Mitochondrial Citrate Carrier, Causes Combined D-2- and L-2-Hydroxyglutaric Aciduria

Nota, B., Struys, E. A., Pop, A., Jansen, E. E., Fernandez Ojeda, M. R., Kanhai, W. A., Kranendijk, M., van Dooren, S. J. M., Bevova, M. R., Sistermans, E. A., et al. (2013) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2013.03.009 · Cited by 138

Mutant IDH1 Confers an in Vivo Growth in a Melanoma Cell Line with BRAF Mutation

Shibata, T., Kokubu, A., Miyamoto, M., Sasajima, Y., & Yamazaki, N. (2011) · American Journal Of Pathology · doi:10.1016/j.ajpath.2010.12.011 · Cited by 131

The widespread role of non-enzymatic reactions in cellular metabolism

Keller, M. A., Piedrafita, G., & Ralser, M. (2015) · Current Opinion in Biotechnology · doi:10.1016/j.copbio.2014.12.020 · Cited by 134

Metabolic network rewiring of propionate flux compensates vitamin B12 deficiency in C. elegans

Watson, E. V., Olín‐Sandoval, V., Hoy, M. J., Li, C., Louisse, T., Yao, V., Mori, A., Holdorf, A. D., Troyanskaya, O. G., Ralser, M., et al. (2016) · eLife · doi:10.7554/elife.17670 · Cited by 122

Mutant ASXL1 cooperates with BAP1 to promote myeloid leukaemogenesis

Asada, S., Goyama, S., Inoue, D., Shikata, S., Takeda, R., Fukushima, T., Yonezawa, T., Fujino, T., Hayashi, Y., Kawabata, K. C., et al. (2018) · Nature Communications · doi:10.1038/s41467-018-05085-9 · Cited by 132

New Molecular Considerations for Glioma: IDH, ATRX, BRAF, TERT, H3 K27M

Karsy, M. J., Guan, J., Cohen, A. L., Jensen, R. L., & Colman, H. (2017) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-017-0722-5 · Cited by 128

Cardio-onco-metabolism: metabolic remodelling in cardiovascular disease and cancer

Karlstaedt, A., Moslehi, J. J., & de Boer, R. A. (2022) · Nature Reviews Cardiology · doi:10.1038/s41569-022-00698-6 · Cited by 130

A Tale of Two Subunits: How the Neomorphic R132H IDH1 Mutation Enhances Production of αHG

Pietrak, B., Zhao, H., Qi, H., Quinn, C. J., Gao, E. N., Boyer, J. G., Concha, N. O., Brown, K., Duraiswami, C., Wooster, R. F., et al. (2011) · Biochemistry · doi:10.1021/bi200499m · Cited by 112

Metabolic dysregulation in monogenic disorders and cancer — finding method in madness

Erez, A., & DeBerardinis, R. J. (2015) · Nature Reviews Cancer · doi:10.1038/nrc3949 · Cited by 105

NAXE Mutations Disrupt the Cellular NAD(P)HX Repair System and Cause a Lethal Neurometabolic Disorder of Early Childhood

Kremer, L. S., Danhauser, K., Herebian, D. A., Ramadža, D. P., Piekutowska‐Abramczuk, D., Seibt, A., Müller‐Felber, W., Haack, T. B., Płoski, R., Lohmeier, K., et al. (2016) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2016.07.018 · Cited by 101

The multifaceted contribution of α-ketoglutarate to tumor progression: An opportunity to exploit?

Abla, H., Sollazzo, M., Gasparre, G., Iommarini, L., & Porcelli, A. M. (2019) · Seminars in Cell and Developmental Biology · doi:10.1016/j.semcdb.2019.05.031 · Cited by 99

Screen for IDH1, IDH2, IDH3, D2HGDH and L2HGDH Mutations in Glioblastoma

Krell, D., Assoku, M., Galloway, M., Mulholland, P. J., Tomlinson, I. P. M., & Bardella, C. (2011) · PLoS ONE · doi:10.1371/journal.pone.0019868 · Cited by 94

Oncometabolites: A New Paradigm for Oncology, Metabolism, and the Clinical Laboratory

Collins, R. R. J., Patel, K., Putnam, W. C., Kapur, P., & Rakheja, D. (2017) · Clinical Chemistry · doi:10.1373/clinchem.2016.267666 · Cited by 94

Evolution and Functional Implications of the Tricarboxylic Acid Cycle as Revealed by Phylogenetic Analysis

Cavalcanti, J. H. F., Esteves-Ferreira, A. A., Quinhones, C. G. S., Pereira-Lima, Í. A., Nunes‐Nesi, A., Fernie, A. R., & Araújo, W. L. (2014) · Genome Biology and Evolution · doi:10.1093/gbe/evu221 · Cited by 91

Metabolic dependencies and vulnerabilities in leukemiaPreprint

Rashkovan, M., & Ferrando, A. A. (2019) · Genes & Development · doi:10.1101/gad.326470.119 · Cited by 92

D-2-hydroxyglutarate produced by mutant IDH2 causes cardiomyopathy and neurodegeneration in micePreprint

Akbay, E. A., Moslehi, J. J., Christensen, C. L., Saha, S. K., Tchaicha, J. H., Ramkissoon, S. H., Stewart, K. M., Carretero, J., Kikuchi, E., Zhang, H., et al. (2014) · Genes & Development · doi:10.1101/gad.231233.113 · Cited by 93

Obstacles to Glioblastoma Treatment Two Decades after Temozolomide

Cruz, J. V. R., Batista, C., Afonso, B. D. H., Alexandre‐Moreira, M. S., Dubois, L. G., Pontes, B., Moura‐Neto, V., & Mendes, F. A. (2022) · Cancers · doi:10.3390/cancers14133203 · Cited by 92

Genetically-defined metabolic reprogramming in cancer

Mullen, A. R., & DeBerardinis, R. J. (2012) · Trends in Endocrinology and Metabolism · doi:10.1016/j.tem.2012.06.009 · Cited by 90

D2HGDH regulates alpha-ketoglutarate levels and dioxygenase function by modulating IDH2

Lin, A., Abbas, S., Kim, S., Ortega, M. M., Bouamar, H., Escobedo, Y. A., Varadarajan, P., Qin, Y., Sudderth, J., Schulz, E., et al. (2015) · Nature Communications · doi:10.1038/ncomms8768 · Cited by 88

Friend or foe—IDH1 mutations in glioma 10 years on

Huang, L. E. (2019) · Carcinogenesis · doi:10.1093/carcin/bgz134 · Cited by 84

The Impact of Non-Enzymatic Reactions and Enzyme Promiscuity on Cellular Metabolism during (Oxidative) Stress Conditions

Piedrafita, G., Keller, M. A., & Ralser, M. (2015) · Biomolecules · doi:10.3390/biom5032101 · Cited by 86

D-2-Hydroxyglutarate in Glioma Biology

Chou, F.-J., Liu, Y., Lang, F., & Yang, C. (2021) · Cells · doi:10.3390/cells10092345 · Cited by 87

Mutations in the Isocitrate Dehydrogenase Genes IDH1 and IDH2 in Tumors

Schaap, F. G., French, P. J., & Bovée, J. V. M. G. (2012) · Advances in Anatomic Pathology · doi:10.1097/pap.0b013e31827b654d · Cited by 80

Saccharomyces cerevisiae Forms d-2-Hydroxyglutarate and Couples Its Degradation to d-Lactate Formation via a Cytosolic Transhydrogenase

Becker‐Kettern, J., Paczia, N., Conrotte, J., Kay, D. P., Guignard, C., Jung, P. P., & Linster, C. L. (2016) · Journal of Biological Chemistry · doi:10.1074/jbc.m115.704494 · Cited by 76

Frontiers in metabolic reconstruction and modeling of plant genomes

Seaver, S. M. D., Henry, C. S., & Hanson, A. D. (2012) · Journal of Experimental Botany · doi:10.1093/jxb/err371 · Cited by 72

Biology of IDH mutant cholangiocarcinoma

Wu, M., Shi, L., Merritt, J., Zhu, A. X., & Bardeesy, N. M. (2022) · Hepatology · doi:10.1002/hep.32424 · Cited by 71

Altered cancer cell metabolism in gliomas with mutant IDH1 or IDH2

Borodovsky, A., Seltzer, M. J., & Riggins, G. J. (2011) · Current Opinion in Oncology · doi:10.1097/cco.0b013e32834d816a · Cited by 71

Metabolite proofreading, a neglected aspect of intermediary metabolism

SCHAFTINGEN, E. V., Rim, R., Marbaix, A., Collard, F., Veiga‐da‐Cunha, M., & Linster, C. L. (2013) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-012-9571-1 · Cited by 67

Increased glutarate production by blocking the glutaryl-CoA dehydrogenation pathway and a catabolic pathway involving l-2-hydroxyglutarate

Zhang, M., Gao, C., Guo, X., Guo, S., Kang, Z., Xiao, D., Yan, J., Tao, F., Zhang, W., Dong, W., et al. (2018) · Nature Communications · doi:10.1038/s41467-018-04513-0 · Cited by 66

Enzymatic assay for quantitative analysis of (d)-2-hydroxyglutarate

Balß, J., Pusch, S., Beck, A.-C., Herold‐Mende, C. C., Krämer, A., Thiede, C., Buckel, W., Langhans, C., Okun, J. G., & von Deimling, A. (2012) · Acta Neuropathologica · doi:10.1007/s00401-012-1060-y · Cited by 66

IDH Mutations in Tumorigenesis and Their Potential Role As Novel Therapeutic Targets

Krell, D., Mulholland, P. J., Frampton, A. E., Krell, J., Stebbing, J., & Bardella, C. (2013) · Future Oncology · doi:10.2217/fon.13.143 · Cited by 63

Detection of 2‐Hydroxyglutarate in Formalin‐Fixed Paraffin‐Embedded Glioma Specimens by Gas Chromatography/Mass Spectrometry

Sahm, F., Capper, D., Pusch, S., Balß, J., Koch, A., Langhans, C., Okun, J. G., & von Deimling, A. (2011) · Brain Pathology · doi:10.1111/j.1750-3639.2011.00506.x · Cited by 61

Current concepts in organic acidurias: understanding intra‐ and extracerebral disease manifestation

Kölker, S., Burgard, P., Sauer, S. W., & Okun, J. G. (2013) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-013-9600-8 · Cited by 62

From genomics to the clinic: biological and translational insights of mutant IDH1/2 in glioma

Dunn, G. P., Andronesi, O. C., & Cahill, D. P. (2013) · Neurosurgical FOCUS · doi:10.3171/2012.12.focus12355 · Cited by 59

Increased mitochondrial activity in a novel IDH1-R132H mutant human oligodendroglioma xenograft model: in situ detection of 2-HG and α-KG

Navis, A. C., Niclou, S. P., Fack, F., Stieber, D., van Lith, S. A. M., Verrijp, K. N., Wright, A. J., Stauber, J., Tops, B. B. J., Otte‐Höller, I., et al. (2013) · Acta Neuropathologica Communications · doi:10.1186/2051-5960-1-18 · Cited by 61

Metabolic adaptations in cancers expressing isocitrate dehydrogenase mutations

Hvinden, I. C., Cadoux-Hudson, T. A. D., Schofield, C. J., & McCullagh, J. (2021) · Cell Reports Medicine · doi:10.1016/j.xcrm.2021.100469 · Cited by 63

The paradox of cancer genes in non-malignant conditions: implications for precision medicine

Adashek, J. J., Kato, S., Lippman, S. M., & Kurzrock, R. (2020) · Genome Medicine · doi:10.1186/s13073-020-0714-y · Cited by 59

Impaired TCA cycle flux in mitochondria in skeletal muscle from type 2 diabetic subjects: Marker or maker of the diabetic phenotype?

Gaster, M., Nehlin, J. O., & Minet, A. D. (2012) · Archives of Physiology and Biochemistry · doi:10.3109/13813455.2012.656653 · Cited by 55

Diffusely infiltrating astrocytomas: pathology, molecular mechanisms and markers

Ichimura, K., Narita, Y., & Hawkins, C. E. (2015) · Acta Neuropathologica · doi:10.1007/s00401-015-1439-7 · Cited by 56

Clinical review of genetic epileptic encephalopathies

Noh, G. J., Asher, Y. J. T., & Graham, J. M. (2012) · European Journal of Medical Genetics · doi:10.1016/j.ejmg.2011.12.010 · Cited by 52

Isocitrate dehydrogenase mutations: A challenge to traditional views on the genesis and malignant progression of gliomas

Weller, M., Wick, W., & von Deimling, A. (2011) · Glia · doi:10.1002/glia.21130 · Cited by 50

Wild-Type IDH Enzymes as Actionable Targets for Cancer Therapy

Bergaggio, E., & Piva, R. (2019) · Cancers · doi:10.3390/cancers11040563 · Cited by 52

A feedback loop engaging propionate catabolism intermediates controls mitochondrial morphology

Zhou, J., Duan, M., Wang, X., Zhang, F., Zhou, H., Ma, T., Yin, Q., Zhang, J., Tian, F., Wang, G., et al. (2022) · Nature Cell Biology · doi:10.1038/s41556-022-00883-2 · Cited by 47

Whole‐exome sequencing detects somatic mutations of IDH1 in metaphyseal chondromatosis with D ‐2‐hydroxyglutaric aciduria (MC‐HGA)

Vissers, L. E. L. M., Fano, V., Martinelli, D., Campos‐Xavier, B., Barbuti, D., Cho, T., Dursun, A., Kim, O. H., Lee, S. H., Timpani, G., et al. (2011) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.34325 · Cited by 49

Roles of IDH1/2 and TET2 mutations in myeloid disorders

Inoue, S., Lemonnier, F., & Mak, T. W. (2016) · International Journal of Hematology · doi:10.1007/s12185-016-1973-7 · Cited by 52

Isocitrate dehydrogenase mutations in diffuse gliomas: clinical and aetiological implications

Gupta, R., Webb-Myers, R., Flanagan, S., & Buckland, M. E. (2011) · Journal of Clinical Pathology · doi:10.1136/jclinpath-2011-200227 · Cited by 49

Computational approach to unravel the impact of missense mutations of proteins (D2HGDH and IDH2) causing D-2-hydroxyglutaric aciduria 2

Kumar, D. T., Emerald, L. J., Doss, C. G. P., Sneha, P., Ramamoorthy, S., Walter, C. E. J., & Zayed, H. (2018) · Metabolic Brain Disease · doi:10.1007/s11011-018-0278-3 · Cited by 48

Cardiac Complications of Propionic and Other Inherited Organic Acidemias

Park, K. C., Krywawych, S., Richard, E., Desviat, L. R., & Swietach, P. (2020) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2020.617451 · Cited by 48

Cardio-Oncology

Karlstaedt, A., Barrett, M., Hu, R., Gammon, S. T., & Ky, B. (2021) · JACC Basic to Translational Science · doi:10.1016/j.jacbts.2021.05.008 · Cited by 48

The oncometabolite D-2-hydroxyglutarate induced by mutant IDH1 or -2 blocks osteoblast differentiation in vitro and in vivo

Suijker, J., Baelde, H. J., Roelofs, H., Cleton-Jansen, A., & Bovée, J. V. M. G. (2015) · Oncotarget · doi:10.18632/oncotarget.4024 · Cited by 47

Impact of CDKN2A/B Homozygous Deletion on the Prognosis and Biology of IDH-Mutant Glioma

Huang, L. E. (2022) · Biomedicines · doi:10.3390/biomedicines10020246 · Cited by 43

Germline gain-of-function mutations of ALK disrupt central nervous system development

de Pontual, L., Kettaneh, D., Gordon, C. T., Oufadem, M., Boddaert, N., Lees, M. M., Balu, L., Lachassinne, E., Petros, A., Mollet, J., et al. (2011) · Human Mutation · doi:10.1002/humu.21442 · Cited by 42

Mitochondrial 2-hydroxyglutarate metabolism

Engqvist, M. K. M., Eßer, C., Maier, A., Lercher, M. J., & Maurino, V. G. (2014) · Mitochondrion · doi:10.1016/j.mito.2014.02.009 · Cited by 46

L2hgdh Deficiency Accumulates l -2-Hydroxyglutarate with Progressive Leukoencephalopathy and Neurodegeneration

Ma, S., Sun, R., Jiang, B., Gao, J., Deng, W., Liu, P., He, R., Cui, J., Ji, M., Yi, W., et al. (2017) · Molecular and Cellular Biology · doi:10.1128/mcb.00492-16 · Cited by 39

Metabolic Drivers in Hereditary Cancer Syndromes

Sciacovelli, M., Schmidt, C., Maher, E. R., & Frezza, C. (2019) · Annual Review of Cancer Biology · doi:10.1146/annurev-cancerbio-030419-033612 · Cited by 39

Mutant IDH1 is required for IDH1 mutated tumor cell growth

Jin, G., Pirozzi, C. J., Chen, L. H., Lopez, G. Y., Duncan, C. G., Feng, J., Spasojević, I., Bigner, D. D., He, Y., & Yan, H. (2012) · Oncotarget · doi:10.18632/oncotarget.577 · Cited by 37

Unraveling the mystery of cancer metabolism in the genesis of tumor-initiating cells and development of cancer

Zhang, G., Yang, P., Guo, P.-D., Miele, L., Sarkar, F. H., Wang, Z. P., & Zhou, Q. (2013) · Biochimica et Biophysica Acta (BBA) - Reviews on Cancer · doi:10.1016/j.bbcan.2013.03.001 · Cited by 34

Enzyme promiscuity, metabolite damage, and metabolite damage control systems of the tricarboxylic acid cycle

Niehaus, T. D., & Hillmann, K. B. (2020) · FEBS Journal · doi:10.1111/febs.15284 · Cited by 34

Structure, substrate specificity, and catalytic mechanism of human D-2-HGDH and insights into pathogenicity of disease-associated mutations

Yang, J., Zhu, H., Zhang, T., & Ding, J. (2021) · Cell Discovery · doi:10.1038/s41421-020-00227-0 · Cited by 32

Pheochromocytomas and Paragangliomas: Bypassing Cellular Respiration

Cascón, A., Remacha, L., Calsina, B., & Robledo, M. (2019) · Cancers · doi:10.3390/cancers11050683 · Cited by 33

Metabolic Syndromes and Malignant Transformation: Where the Twain Shall Meet

Bhagwat, N., & Levine, R. L. (2010) · Science Translational Medicine · doi:10.1126/scitranslmed.3001669 · Cited by 4

Cancer-associated IDH1 mutations produce 2-hydroxyglutarate

Dang, L., White, D. W., Gross, S., Bennett, B. D., Bittinger, M. A., Driggers, E. M., Fantin, V. R., Jang, H. G., Jin, S., Keenan, M. C., et al. (2010) · Nature · doi:10.1038/nature09132 · Cited by 613

Mutations in the d-2-Hydroxyglutarate Dehydrogenase Gene Cause d-2-Hydroxyglutaric Aciduria

Struys, E. A., Salomons, G. S., Achouri, Y., Van Schaftingen, E., Grosso, S., Craigen, W. J., Verhoeven, N. M., & Jakobs, C. (2005) · The American Journal of Human Genetics · doi:10.1086/427890 · Cited by 172

Isocitrate dehydrogenase 2 (IDH2)

(2010) · Science-Business eXchange · doi:10.1038/scibx.2010.1188

IDH1 and IDH2 have critical roles in 2-hydroxyglutarate production in D-2-hydroxyglutarate dehydrogenase depleted cells

Matsunaga, H., Futakuchi-Tsuchida, A., Takahashi, M., ISHIKAWA, T., Tsuji, M., & Ando, O. (2012) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2012.06.002 · Cited by 28