[1]JournaloftheAmericanChemicalSociety,1956,vol.78,p.2423,2428
[2]TetrahedronLetters,1995,vol.36,p.1637-1640
[3]ChemischeBerichte,1912,vol.45,p.2449
[4]BioorganicandMedicinalChemistryLetters,2012,vol.22,p.482-484
[1]ChemischeBerichte,1912,vol.45,p.2449
[2]ChemischeBerichte,1912,vol.45,p.2449
[3]HelveticaChimicaActa,1919,vol.2,p.449
[1]BiochemischeZeitschrift,1931,vol.232,p.435,437,438
[2]BiochemischeZeitschrift,1930,vol.225,p.244
[3]BiochemischeZeitschrift,1931,vol.236,p.241,245
[4]BiochemischeZeitschrift,1931,vol.233,p.370
13095-47-1
[1]RocznikiChemii,1975,vol.49,p.151-157
[1]AgriculturalandBiologicalChemistry,1983,vol.47,p.2619-2624
[2]AgriculturalandBiologicalChemistry,1983,vol.47,p.2619-2624
Title: Enasidenib induces acute myeloid leukemia cell differentiation to promote clinical response.
Journal: Blood 20170810
Title: Experimental evidence of oxidative stress in patients with l-2-hydroxyglutaric aciduria and that l-carnitine attenuates in vitro DNA damage caused by d-2-hydroxyglutaric and l-2-hydroxyglutaric acids.
Journal: Toxicology in vitro : an international journal published in association with BIBRA 20170801
Title: Oncometabolites d- and l-2-Hydroxyglutarate Inhibit the AlkB Family DNA Repair Enzymes under Physiological Conditions.
Journal: Chemical research in toxicology 20170417
Title: Plasma 2-hydroxyglutarate and hexanoylcarnitine levels are potential biomarkers for skeletal muscle toxicity in male Fischer 344 rats.
Journal: The Journal of toxicological sciences 20170101
Title: Inhibition of mutant IDH1 decreases D-2-HG levels without affecting tumorigenic properties of chondrosarcoma cell lines.
Journal: Oncotarget 20150520
Title: Rotenone Stereospecifically Increases (S)-2-Hydroxyglutarate in SH-SY5Y Neuronal Cells.
Journal: Chemical research in toxicology 20150518
Title: An inhibitor of mutant IDH1 delays growth and promotes differentiation of glioma cells.
Journal: Science (New York, N.Y.) 20130503
Title: Development of a satisfactory and general continuous assay for aminotransferases by coupling with (R)-2-hydroxyglutarate dehydrogenase.
Journal: Analytical biochemistry 20121215
Title: IDH1 and IDH2 mutations in tumorigenesis: mechanistic insights and clinical perspectives.
Journal: Clinical cancer research : an official journal of the American Association for Cancer Research 20121015
Title: Detection of 'oncometabolite' 2-hydroxyglutarate by magnetic resonance analysis as a biomarker of IDH1/2 mutations in glioma.
Journal: Journal of molecular medicine (Berlin, Germany) 20121001
Title: 4-Hydroxy-2-oxoglutarate aldolase inactivity in primary hyperoxaluria type 3 and glyoxylate reductase inhibition.
Journal: Biochimica et biophysica acta 20121001
Title: D-2-hydroxyglutarate produced by mutant IDH1 perturbs collagen maturation and basement membrane function.
Journal: Genes & development 20120915
Title: IDH1(R132H) mutation increases murine haematopoietic progenitors and alters epigenetics.
Journal: Nature 20120830
Title: 2-Hydroxyglutarate concentration in serum from patients with gliomas does not correlate with IDH1/2 mutation status or tumor size.
Journal: International journal of cancer 20120801
Title: IDH1 and IDH2 have critical roles in 2-hydroxyglutarate production in D-2-hydroxyglutarate dehydrogenase depleted cells.
Journal: Biochemical and biophysical research communications 20120706
Title: Identification of additional IDH mutations associated with oncometabolite R(-)-2-hydroxyglutarate production.
Journal: Oncogene 20120510
Title: 2-hydroxyglutarate detection by magnetic resonance spectroscopy in IDH-mutated patients with gliomas.
Journal: Nature medicine 20120401
Title: Transformation by the (R)-enantiomer of 2-hydroxyglutarate linked to EGLN activation.
Journal: Nature 20120322
Title: Non-invasive detection of 2-hydroxyglutarate and other metabolites in IDH1 mutant glioma patients using magnetic resonance spectroscopy.
Journal: Journal of neuro-oncology 20120301
Title: IDH mutation impairs histone demethylation and results in a block to cell differentiation.
Journal: Nature 20120215
Title: L-2-hydroxyglutaric aciduria diagnosed in a young adult with progressive cerebellar ataxia and facial dyskinesia.
Journal: Revue neurologique 20120201
Title: Detection of 2-hydroxyglutarate in IDH-mutated glioma patients by in vivo spectral-editing and 2D correlation magnetic resonance spectroscopy.
Journal: Science translational medicine 20120111
Title: Magnetic resonance of 2-hydroxyglutarate in IDH1-mutated low-grade gliomas.
Journal: Science translational medicine 20120111
Title: Detection of 2-hydroxyglutarate in formalin-fixed paraffin-embedded glioma specimens by gas chromatography/mass spectrometry.
Journal: Brain pathology (Zurich, Switzerland) 20120101
Title: Production of 2-hydroxyglutarate by isocitrate dehydrogenase 1-mutated gliomas: an evolutionary alternative to the Warburg shift?
Journal: Neuro-oncology 20111201
Title: A lymphoblast model for IDH2 gain-of-function activity in d-2-hydroxyglutaric aciduria type II: novel avenues for biochemical and therapeutic studies.
Journal: Biochimica et biophysica acta 20111101
Title: Whole-exome sequencing detects somatic mutations of IDH1 in metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria (MC-HGA).
Journal: American journal of medical genetics. Part A 20111101
Title: Mutations to metabolic enzymes in cancer herald a need to unify genetics and biochemistry.
Journal: Cell cycle (Georgetown, Tex.) 20110901
Title: Glutamate racemization and catabolism in Fusobacterium varium.
Journal: The FEBS journal 20110701
Title: A tale of two subunits: how the neomorphic R132H IDH1 mutation enhances production of αHG.
Journal: Biochemistry 20110531
Title: The oncometabolite 2-hydroxyglutarate inhibits histone lysine demethylases.
Journal: EMBO reports 20110501
Title: Papillary thyroid carcinoma shows elevated levels of 2-hydroxyglutarate.
Journal: Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine 20110401
Title: The sodium-dependent di- and tricarboxylate transporter, NaCT, is not responsible for the uptake of D-, L-2-hydroxyglutarate and 3-hydroxyglutarate into neurons.
Journal: Journal of inherited metabolic disease 20110401
Title: Plant D-2-hydroxyglutarate dehydrogenase participates in the catabolism of lysine especially during senescence.
Journal: The Journal of biological chemistry 20110401
Title: Isocitrate dehydrogenase 1/2 mutational analyses and 2-hydroxyglutarate measurements in Wilms tumors.
Journal: Pediatric blood & cancer 20110301
Title: Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases.
Journal: Cancer cell 20110118
Title: 2-hydroxyglutarate production, but not dominant negative function, is conferred by glioma-derived NADP-dependent isocitrate dehydrogenase mutations.
Journal: PloS one 20110101
Title: Cancer-associated IDH mutations: biomarker and therapeutic opportunities.
Journal: Oncogene 20101209
Title: Analysis of isocitrate dehydrogenase-1/2 gene mutations in gliomas.
Journal: Chinese medical journal 20101201
Title: Inhibition of glutaminase preferentially slows growth of glioma cells with mutant IDH1.
Journal: Cancer research 20101115
Title: Increased levels of 2-hydroxyglutarate in AML patients with IDH1-R132H and IDH2-R140Q mutations.
Journal: European journal of haematology 20101101
Title: Metabolic syndromes and malignant transformation: where the twain shall meet.
Journal: Science translational medicine 20101020
Title: IDH2 mutations in patients with D-2-hydroxyglutaric aciduria.
Journal: Science (New York, N.Y.) 20101015
Title: Metabolic pathway of α-ketoglutarate in Lactobacillus sanfranciscensis and Lactobacillus reuteri during sourdough fermentation.
Journal: Journal of applied microbiology 20101001
Title: Isocitrate dehydrogenase 1 and 2 mutations in cancer: alterations at a crossroads of cellular metabolism.
Journal: Journal of the National Cancer Institute 20100707
Title: Oncometabolite? IDH1 discoveries raise possibility of new metabolism targets in brain cancers and leukemia.
Journal: Journal of the National Cancer Institute 20100707
Title: Novel biosynthesis of (R)-ethyl-3-hydroxyglutarate with (R)-enantioselective hydrolysis of racemic ethyl 4-cyano-3-hydroxybutyate by Rhodococcus erythropolis.
Journal: Applied microbiology and biotechnology 20100701
Title: Cancer-associated IDH1 mutations produce 2-hydroxyglutarate.
Journal: Nature 20100617
Title: L-2-hydroxyglutaric aciduria and brain tumors.
Journal: Journal of pediatric hematology/oncology 20100501
Title: IDH1 and IDH2 mutations in gliomas and the associated induction of hypoxia-inducible factor and production of 2-hydroxyglutarate.
Journal: Neurosurgery 20100401
Title: 2-hydroxyglutarate accumulation caused by IDH mutation is involved in the formation of malignant gliomas.
Journal: Expert review of neurotherapeutics 20100401
Title: Metabolism, gliomas, and IDH1.
Journal: The New England journal of medicine 20100325
Title: The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate to 2-hydroxyglutarate.
Journal: Cancer cell 20100316
Title: Evidence for genetic heterogeneity in D-2-hydroxyglutaric aciduria.
Journal: Human mutation 20100301
Title: L-2-hydroxyglutaric aciduria and multiple acyl-coenzyme A dehydrogenase deficiency are distinct metabolic disorders.
Journal: Journal of pediatric hematology/oncology 20100301
Title: L-2-hydroxyglutaric aciduria diagnosed in an adult presenting with acute deterioration.
Journal: Journal of neurology 20100101
Title: Cancer-associated IDH1 mutations produce 2-hydroxyglutarate.
Journal: Nature 20091210
Title: [D-2-hydroxyglutaric aciduria. Report of two cases].
Journal: Investigacion clinica 20090901
Title: Sudden unexpected death in an infant with L-2-hydroxyglutaric aciduria.
Journal: European journal of pediatrics 20090801
Title: L-2-Hydroxyglutaric aciduria: pattern of MR imaging abnormalities in 56 patients.
Journal: Radiology 20090601
Title: [L-2 hydroxyglutaric aciduria: presentation of a family diagnosed in adulthood].
Journal: Neurologia (Barcelona, Spain) 20090601
Title: L: -2-Hydroxyglutaric aciduria, a disorder of metabolite repair.
Journal: Journal of inherited metabolic disease 20090401
Title: Measurement of D: -2-hydroxyglutarate dehydrogenase activity in cell homogenates derived from D: -2-hydroxyglutaric aciduria patients.
Journal: Journal of inherited metabolic disease 20090401
Title: Riboflavin treatment in a case with l-2-hydroxyglutaric aciduria.
Journal: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 20090101
Title: Tracing the origin of L-2-hydroxyglutaric aciduria in a family.
Journal: The International journal of neuroscience 20090101
Title: L-2-hydroxyglutaric aciduria: identification of ten novel mutations in the L2HGDH gene.
Journal: Journal of inherited metabolic disease 20081201
Title: Organic anion transporters OAT1 and OAT4 mediate the high affinity transport of glutarate derivatives accumulating in patients with glutaric acidurias.
Journal: Pflugers Archiv : European journal of physiology 20081001
Title: L-2-Hydroxyglutaric aciduria presenting with severe autistic features.
Journal: Brain & development 20080401
Title: L-2-hydroxyglutaric aciduria and brain tumors in children with mutations in the L2HGDH gene: neuroimaging findings.
Journal: Neuropediatrics 20080401
Title: Investigation of a wide spectrum of inherited metabolic disorders by 13C NMR spectroscopy.
Journal: Acta biochimica Polonica 20080101
Title: Neuropathological findings in a Staffordshire bull terrier with l-2-hydroxyglutaric aciduria.
Journal: Journal of comparative pathology 20080101
Title: Osteoma of the calvaria in L-2-hydroxyglutaric aciduria.
Journal: Journal of inherited metabolic disease 20071101
Title: L-2-hydroxyglutaric aciduria, a defect of metabolite repair.
Journal: Journal of inherited metabolic disease 20071001
Title: Novel insights into L-2-hydroxyglutaric aciduria: mass isotopomer studies reveal 2-oxoglutaric acid as the metabolic precursor of L-2-hydroxyglutaric acid.
Journal: Journal of inherited metabolic disease 20071001
Title: Alpha-ketoglutarate is the precursor of L-2-hydroxyglutarate.
Journal: Journal of inherited metabolic disease 20071001
Title: Mutation analysis a prerequisite for prenatal diagnosis of L-2-hydroxyglutaric aciduria?
Journal: Molecular genetics and metabolism 20070801
Title: L-2-hydroxyglutaric aciduria: characterisation of the molecular defect in a spontaneous canine model.
Journal: Journal of medical genetics 20070501
Title: Toward an improved understanding of the glutamate mutase system.
Journal: Journal of the American Chemical Society 20070214
Title: Neurotoxicity assessment using zebrafish.
Journal: Journal of pharmacological and toxicological methods 20070101
Title: [L-2-hydroxyglutaric aciduria, an error of metabolism].
Journal: Bulletin et memoires de l'Academie royale de medecine de Belgique 20070101
Title: Case 2: Suspected non-accidental injury.
Journal: Acta paediatrica (Oslo, Norway : 1992) 20061001
Title: Normal pregnancy outcome in L-2-hydroxyglutaric aciduria.
Journal: Journal of inherited metabolic disease 20060801
Title: Hemiconvulsion-hemiplegia-epilepsy syndrome as a presenting feature of L-2-hydroxyglutaric aciduria.
Journal: Journal of child neurology 20060601
Title: D-2-hydroxyglutaric aciduria in three patients with proven SSADH deficiency: genetic coincidence or a related biochemical epiphenomenon?
Journal: Molecular genetics and metabolism 20060501
Title: L-2-Hydroxyglutaric aciduria: clinical, genetic, and brain MRI characteristics in two adult sisters.
Journal: European journal of neurology 20060501
Title: L-2-hydroxyglutaric aciduria: a report of six cases and review of the literature.
Journal: Archives of Iranian medicine 20060401
Title: Metabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase.
Journal: Metabolism: clinical and experimental 20060301
Title: D-2-Hydroxyglutaric aciduria: unravelling the biochemical pathway and the genetic defect.
Journal: Journal of inherited metabolic disease 20060201
Title: The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase.
Journal: Biochimie 20060101
Title: [L-2-hydroxyglutaric aciduria -- a rare cause of macrocephaly].
Journal: Klinische Padiatrie 20060101
Title: A zebrafish assay for identifying neuroprotectants in vivo.
Journal: Neurotoxicology and teratology 20060101
Title: Electronic structure studies of the adenosylcobalamin cofactor in glutamate mutase.
Journal: Biochemistry 20051122
Title: Novel L2HGDH mutations in 21 patients with L-2-hydroxyglutaric aciduria of Portuguese origin.
Journal: Human mutation 20051001
Title: L-2-hydroxyglutaric aciduria: clinical, neuroimaging, and neuropathological findings.
Journal: Archives of neurology 20050401
Title: Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause D-2-hydroxyglutaric aciduria.
Journal: American journal of human genetics 20050201
Title: L-2-hydroxyglutaric aciduria in a West Highland white terrier.
Journal: The Veterinary record 20050129
Title: Metaphyseal enchondrodysplasia with 2-hydroxy-glutaric aciduria: observation of a third case and further delineation.
Journal: Clinical dysmorphology 20050101
Title: Structural basis for stereo-specific catalysis in NAD(+)-dependent (R)-2-hydroxyglutarate dehydrogenase from Acidaminococcus fermentans.
Journal: The FEBS journal 20050101
Title: L-2-hydroxyglutaric aciduria: a report of 29 patients.
Journal: The Turkish journal of pediatrics 20050101
Title: Mitochondrial energy metabolism is markedly impaired by D-2-hydroxyglutaric acid in rat tissues.
Journal: Molecular genetics and metabolism 20050101
Title: Phenotypic heterogeneity in the presentation of D-2-hydroxyglutaric aciduria in monozygotic twins.
Journal: Molecular genetics and metabolism 20050101
Title: Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias.
Journal: Journal of inherited metabolic disease 20050101
Title: Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D- and L-2 hydroxyglutaric aciduria.
Journal: Journal of inherited metabolic disease 20050101
Title: Sodium ion pumps and hydrogen production in glutamate fermenting anaerobic bacteria.
Journal: Journal of molecular microbiology and biotechnology 20050101
Title: A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria.
Journal: Proceedings of the National Academy of Sciences of the United States of America 20041130
Title: L-2-Hydroxyglutaric aciduria: identification of a mutant gene C14orf160, localized on chromosome 14q22.1.
Journal: Human molecular genetics 20041115
Title: Measurement of urinary D- and L-2-hydroxyglutarate enantiomers by stable-isotope-dilution liquid chromatography-tandem mass spectrometry after derivatization with diacetyl-L-tartaric anhydride.
Journal: Clinical chemistry 20040801
Title: [L-2-hydroxyglutaric aciduria: clinical, biochemical and neuroradiological findings in two Venezuelan patients].
Journal: Revista de neurologia 20040801
Title: Identification of a dehydrogenase acting on D-2-hydroxyglutarate.
Journal: The Biochemical journal 20040701
Title: D-2-hydroxyglutaric aciduria and glutaric aciduria type 1 in siblings: coincidence, or linked disorders?
Journal: Neuropediatrics 20040601
Title: L-2-hydroxyglutaric aciduria and brain malignant tumors: a predisposing condition?
Journal: Neurology 20040525
Title: In vitro effects of D-2-hydroxyglutaric acid on glutamate binding, uptake and release in cerebral cortex of rats.
Journal: Journal of the neurological sciences 20040215
Title: Investigations by mass isotopomer analysis of the formation of D-2-hydroxyglutarate by cultured lymphoblasts from two patients with D-2-hydroxyglutaric aciduria.
Journal: FEBS letters 20040116
Title: Inhibition of creatine kinase activity from rat cerebral cortex by D-2-hydroxyglutaric acid in vitro.
Journal: Neurochemistry international 20040101
Title: Single-voxel MR spectroscopy and diffusion-weighted MRI in two patients with l-2-hydroxyglutaric aciduria.
Journal: Pediatric radiology 20031201
Title: D-2-hydroxyglutaric acid inhibits creatine kinase activity from cardiac and skeletal muscle of young rats.
Journal: European journal of clinical investigation 20031001
Title: Protein farnesyltransferase inhibitors interfere with farnesyl diphosphate binding by rubber transferase.
Journal: European journal of biochemistry 20031001
Title: Inhibition of mitochondrial creatine kinase activity by D-2-hydroxyglutaric acid in cerebellum of young rats.
Journal: Neurochemical research 20030901
Title: Effects of L-2-hydroxyglutaric acid on various parameters of the glutamatergic system in cerebral cortex of rats.
Journal: Metabolic brain disease 20030901
Title: D-2-hydroxyglutaric aciduria: a case with an intermediate phenotype and prenatal diagnosis of two affected fetuses.
Journal: American journal of medical genetics. Part A 20030801
Title: L-2-hydroxyglutaric aciduria in Staffordshire bull terriers.
Journal: The Veterinary record 20030719
Title: Disease-related metabolites in culture medium of fibroblasts from patients with D-2-hydroxyglutaric aciduria, L-2-hydroxyglutaric aciduria, and combined D/L-2-hydroxyglutaric aciduria.
Journal: Clinical chemistry 20030701
Title: L-2-hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats.
Journal: International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience 20030601
Title: Regulation of the mitochondrial ATP-synthase in health and disease.
Journal: Molecular genetics and metabolism 20030601
Title: D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats.
Journal: The European journal of neuroscience 20030501
Title: L-2 hydroxyglutaric aciduria: proton magnetic resonance spectroscopy and diffusion magnetic resonance imaging findings.
Journal: Journal of computer assisted tomography 20030101
Title: D-2-Hydroxyglutaric aciduria with absence of corpus callosum and neonatal intracranial haemorrhage.
Journal: Journal of inherited metabolic disease 20030101
Title: Determination of the absolute configuration of 2-hydroxyglutaric acid and 5-oxoproline in urine samples by high-resolution NMR spectroscopy in the presence of chiral lanthanide complexes.
Journal: Journal of pharmaceutical and biomedical analysis 20020615
Title: D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings.
Journal: Journal of inherited metabolic disease 20020201
Title: Inhibition of cytochrome c oxidase activity in rat cerebral cortex and human skeletal muscle by D-2-hydroxyglutaric acid in vitro.
Journal: Biochimica et biophysica acta 20020102
Title: D-2-Hydroxyglutaric aciduria and subdural haemorrhage.
Journal: Acta paediatrica (Oslo, Norway : 1992) 20020101
Title: L-2-hydroxyglutaric aciduria presenting as migraine.
Journal: Neurology 20011113
Title: A Korean girl with alpha-aminoadipic and alpha-ketoadipic aciduria accompanied with elevation of 2-hydroxyglutarate and glutarate.
Journal: Journal of inherited metabolic disease 20010801
Title: Biosynthesis of methanopterin.
Journal: Biochemistry 19960319
Title: Xu W, et al. Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases. Cancer Cell. 2011 Jan 18;19(1):17-30.
Title: Fu X, et al. 2-Hydroxyglutarate Inhibits ATP Synthase and mTOR Signaling. Cell Metab. 2015 Sep 1;22(3):508-15.
Title: Martijn Kranendijk, et al. Progress in understanding 2-hydroxyglutaric acidurias. J Inherit Metab Dis. 2012 Jul;35(4):571-87.
Title: Martin Böttcher, et al. D-2-hydroxyglutarate interferes with HIF-1α stability skewing T-cell metabolism towards oxidative phosphorylation and impairing Th17 polarization. Oncoimmunology. 2018 Mar 26;7(7):e1445454.
Title: Alexandra Latini , et al. D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats. Eur J Neurosci. 2003 May;17(10):2017-22.
