Published in J Inherit Metab Dis on June 21, 2007
Cancer-associated metabolite 2-hydroxyglutarate accumulates in acute myelogenous leukemia with isocitrate dehydrogenase 1 and 2 mutations. J Exp Med (2010) 5.68
Inhibition of glutaminase preferentially slows growth of glioma cells with mutant IDH1. Cancer Res (2010) 3.30
What a difference a hydroxyl makes: mutant IDH, (R)-2-hydroxyglutarate, and cancer. Genes Dev (2013) 2.31
Progress in understanding 2-hydroxyglutaric acidurias. J Inherit Metab Dis (2012) 1.78
Oxidation of alpha-ketoglutarate is required for reductive carboxylation in cancer cells with mitochondrial defects. Cell Rep (2014) 1.64
Metabolite damage and its repair or pre-emption. Nat Chem Biol (2013) 1.50
Hypoxia Induces Production of L-2-Hydroxyglutarate. Cell Metab (2015) 1.45
L: -2-Hydroxyglutaric aciduria, a disorder of metabolite repair. J Inherit Metab Dis (2008) 1.40
IDH1 and IDH2 mutations in tumorigenesis: mechanistic insights and clinical perspectives. Clin Cancer Res (2012) 1.36
Hypoxia-Mediated Increases in L-2-hydroxyglutarate Coordinate the Metabolic Response to Reductive Stress. Cell Metab (2015) 1.34
Novel insights into L-2-hydroxyglutaric aciduria: mass isotopomer studies reveal 2-oxoglutaric acid as the metabolic precursor of L-2-hydroxyglutaric acid. J Inherit Metab Dis (2007) 1.27
L-2-Hydroxyglutarate: an epigenetic modifier and putative oncometabolite in renal cancer. Cancer Discov (2014) 1.26
The redox basis of epigenetic modifications: from mechanisms to functional consequences. Antioxid Redox Signal (2011) 1.24
Fundamentals of cancer metabolism. Sci Adv (2016) 1.09
New developments in the pathogenesis and therapeutic targeting of the IDH1 mutation in glioma. Int J Med Sci (2015) 1.00
Metabolite proofreading, a neglected aspect of intermediary metabolism. J Inherit Metab Dis (2013) 0.99
D-2-hydroxyglutarate produced by mutant IDH2 causes cardiomyopathy and neurodegeneration in mice. Genes Dev (2014) 0.97
Applications of NMR spectroscopy to systems biochemistry. Prog Nucl Magn Reson Spectrosc (2016) 0.95
A novel GDP-D-glucose phosphorylase involved in quality control of the nucleoside diphosphate sugar pool in Caenorhabditis elegans and mammals. J Biol Chem (2011) 0.94
Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading. J Biol Chem (2011) 0.91
Mitochondrial Citrate Transporter-dependent Metabolic Signature in the 22q11.2 Deletion Syndrome. J Biol Chem (2015) 0.90
Metabolic consequences of oncogenic IDH mutations. Pharmacol Ther (2015) 0.90
Identification of Escherichia coli YgaF as an L-2-hydroxyglutarate oxidase. J Bacteriol (2008) 0.88
Metabolite proofreading in carnosine and homocarnosine synthesis: molecular identification of PM20D2 as β-alanyl-lysine dipeptidase. J Biol Chem (2014) 0.87
Ultra high throughput sequencing excludes MDH1 as candidate gene for RP28-linked retinitis pigmentosa. Mol Vis (2009) 0.87
A mouse model of L-2-hydroxyglutaric aciduria, a disorder of metabolite repair. PLoS One (2015) 0.87
Evidence that 2-hydroxyglutarate is not readily metabolized in colorectal carcinoma cells. Cancer Metab (2015) 0.86
Homocysteine methyltransferases Mht1 and Sam4 prevent the accumulation of age-damaged (R,S)-AdoMet in the yeast Saccharomyces cerevisiae. J Biol Chem (2010) 0.84
2-Hydroxy Acids in Plant Metabolism. Arabidopsis Book (2015) 0.80
Drosophila larvae synthesize the putative oncometabolite L-2-hydroxyglutarate during normal developmental growth. Proc Natl Acad Sci U S A (2017) 0.79
The Impact of Non-Enzymatic Reactions and Enzyme Promiscuity on Cellular Metabolism during (Oxidative) Stress Conditions. Biomolecules (2015) 0.79
L-2-Hydroxyglutarate production arises from noncanonical enzyme function at acidic pH. Nat Chem Biol (2017) 0.78
A pathway for repair of NAD(P)H in plants. J Biol Chem (2014) 0.78
A L2HGDH initiator methionine codon mutation in a Yorkshire terrier with L-2-hydroxyglutaric aciduria. BMC Vet Res (2012) 0.78
Rotenone Stereospecifically Increases (S)-2-Hydroxyglutarate in SH-SY5Y Neuronal Cells. Chem Res Toxicol (2015) 0.78
Development and implementation of a novel assay for L-2-hydroxyglutarate dehydrogenase (L-2-HGDH) in cell lysates: L-2-HGDH deficiency in 15 patients with L-2-hydroxyglutaric aciduria. J Inherit Metab Dis (2009) 0.78
Saccharomyces cerevisiae Forms d-2-Hydroxyglutarate and Couples Its Degradation to d-Lactate Formation via a Cytosolic Transhydrogenase. J Biol Chem (2016) 0.78
Sudden unexpected death in an infant with L-2-hydroxyglutaric aciduria. Eur J Pediatr (2008) 0.76
Another small molecule in the oncometabolite mix: L-2-Hydroxyglutarate in kidney cancer. Oncoscience (2015) 0.76
Alpha-ketoglutarate is the precursor of L-2-hydroxyglutarate. J Inherit Metab Dis (2007) 0.75
Isocitrate dehydrogenases in physiology and cancer: biochemical and molecular insight. Cell Biosci (2017) 0.75
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal Biochem (1976) 903.81
Tissue fractionation studies. 6. Intracellular distribution patterns of enzymes in rat-liver tissue. Biochem J (1955) 38.61
The redox state of free nicotinamide-adenine dinucleotide in the cytoplasm and mitochondria of rat liver. Biochem J (1967) 12.40
Alpha-hydroxyglutaric acid synthetase. J Bacteriol (1962) 2.50
A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria. Proc Natl Acad Sci U S A (2004) 2.19
Isolation and properties of malic dehydrogenase from ox-heart mitochondria. Biochem J (1957) 2.16
Analytical study of microsomes and isolated subcellular membranes from rat liver. II. Preparation and composition of the microsomal fraction. J Cell Biol (1974) 1.95
Aging as war between chemical and biochemical processes: protein methylation and the recognition of age-damaged proteins for repair. Ageing Res Rev (2003) 1.90
House cleaning, a part of good housekeeping. Mol Microbiol (2006) 1.80
Identification of a dehydrogenase acting on D-2-hydroxyglutarate. Biochem J (2004) 1.72
The oxidation of l(-)alpha-hydroxyglutaric acid in animal tissues. Biochem J (1937) 1.71
Fructosamine 3-kinase is involved in an intracellular deglycation pathway in human erythrocytes. Biochem J (2002) 1.70
L-2-Hydroxyglutaric aciduria: an inborn error of metabolism? J Inherit Metab Dis (1980) 1.67
The formation of methylglyoxal from triose phosphates. Investigation using a specific assay for methylglyoxal. Eur J Biochem (1993) 1.63
L-2-Hydroxyglutaric aciduria: identification of a mutant gene C14orf160, localized on chromosome 14q22.1. Hum Mol Genet (2004) 1.50
Spontaneous tumorigenesis in mice defective in the MTH1 gene encoding 8-oxo-dGTPase. Proc Natl Acad Sci U S A (2001) 1.48
Kinetic parameters for the elimination reaction catalyzed by triosephosphate isomerase and an estimation of the reaction's physiological significance. Biochemistry (1991) 1.20
Deficiency in protein L-isoaspartyl methyltransferase results in a fatal progressive epilepsy. J Neurosci (1998) 1.19
Identification of an archaeal 2-hydroxy acid dehydrogenase catalyzing reactions involved in coenzyme biosynthesis in methanoarchaea. J Bacteriol (2000) 1.18
L-2-hydroxyglutaric acidemia: a novel inherited neurometabolic disease. Ann Neurol (1992) 1.16
Roles of methionine suldfoxide reductases in antioxidant defense, protein regulation and survival. Curr Pharm Des (2005) 1.08
Reduction of alpha-ketoglutarate by homogeneous lactic dehydrogenase X of testicular tissue. J Biol Chem (1969) 1.07
The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase. Biochimie (2005) 1.06
Limited accumulation of damaged proteins in l-isoaspartyl (D-aspartyl) O-methyltransferase-deficient mice. J Biol Chem (2001) 1.05
Dehydrogenase regulation of metabolite oxidation and efflux from mitochondria in intact hearts. Am J Physiol (1998) 1.00
Novel L2HGDH mutations in 21 patients with L-2-hydroxyglutaric aciduria of Portuguese origin. Hum Mutat (2005) 0.97
Incorporation and utilization of [3-13C]lactate and [1,2-13C]acetate by rat skeletal muscle. J Appl Physiol (1985) (1999) 0.95
Equilibrium constants of several reactions involved in the fermentation of glutamate. Eur J Biochem (1987) 0.92
L-2-hydroxyglutaric acidaemia: clinical and biochemical findings in 12 patients and preliminary report on L-2-hydroxyacid dehydrogenase. J Inherit Metab Dis (1993) 0.91
Increased protein glycation in fructosamine 3-kinase-deficient mice. Biochem J (2006) 0.91
Conversion of a synthetic fructosamine into its 3-phospho derivative in human erythrocytes. Biochem J (2000) 0.88
Characterization of the functional role of allosteric site residue Asp102 in the regulatory mechanism of human mitochondrial NAD(P)+-dependent malate dehydrogenase (malic enzyme). Biochem J (2005) 0.86
L-2-Hydroxyglutaric aciduria: neuropathological correlations and first report of severe neurodegenerative disease and neonatal death. J Inherit Metab Dis (1996) 0.85
Mitochondrial metabolism in different thyroid states. Biochem J (1992) 0.83
Alteration of the specificity of malate dehydrogenase by chemical modulation of an active site arginine. J Biol Chem (2001) 0.81
Cerebral metabolic state following complete compression ischemia. Brain Res (1974) 0.81
Rat liver mitochondrial malate dehydrogenase: purification, kinetic properties, and role in ethanol metabolism. Arch Biochem Biophys (1991) 0.77
A kinetic study of pyrophosphate: fructose-6-phosphate phosphotransferase from potato tubers. Application to a microassay of fructose 2,6-bisphosphate. Eur J Biochem (1982) 6.74
Fructose 2,6-bisphosphate 2 years after its discovery. Biochem J (1982) 2.91
Control of the fructose-6-phosphate/fructose 1,6-bisphosphate cycle in isolated hepatocytes by glucose and glucagon. Role of a low-molecular-weight stimulator of phosphofructokinase. Biochem J (1980) 2.88
Fructose 2,6-bisphosphate, the probably structure of the glucose- and glucagon-sensitive stimulator of phosphofructokinase. Biochem J (1980) 2.79
Inhibition of fructose-1,6-bisphosphatase by fructose 2,6-biphosphate. Proc Natl Acad Sci U S A (1981) 2.44
Mutations in PMM2, a phosphomannomutase gene on chromosome 16p13, in carbohydrate-deficient glycoprotein type I syndrome (Jaeken syndrome). Nat Genet (1997) 2.30
A new class of phosphotransferases phosphorylated on an aspartate residue in an amino-terminal DXDX(T/V) motif. J Biol Chem (1998) 2.29
A CCAAT DNA binding factor consisting of two different components that are both required for DNA binding. J Biol Chem (1988) 1.93
Control of liver 6-phosphofructokinase by fructose 2,6-bisphosphate and other effectors. Proc Natl Acad Sci U S A (1981) 1.91
Sequence of a putative glucose 6-phosphate translocase, mutated in glycogen storage disease type Ib. FEBS Lett (1997) 1.86
Fructose 2,6-bisphosphate. Adv Enzymol Relat Areas Mol Biol (1987) 1.71
Study of the fructose 6-phosphate/fructose 1,6-bi-phosphate cycle in the liver in vivo. Biochem J (1980) 1.69
Lack of homozygotes for the most frequent disease allele in carbohydrate-deficient glycoprotein syndrome type 1A. Am J Hum Genet (1998) 1.66
Hormonal control of fructose 2,6-bisphosphate concentration in isolated rat hepatocytes. Biochem J (1983) 1.64
Phosphofructokinase 2: the enzyme that forms fructose 2,6-bisphosphate from fructose 6-phosphate and ATP. Biochem Biophys Res Commun (1981) 1.58
3-Phosphoglycerate dehydrogenase deficiency: an inborn error of serine biosynthesis. Arch Dis Child (1996) 1.45
Effectors of the regulatory protein acting on liver glucokinase: a kinetic investigation. Eur J Biochem (1991) 1.44
Pathway and regulation of erythritol formation in Leuconostoc oenos. J Bacteriol (1993) 1.43
The mechanism by which rat liver glucokinase is inhibited by the regulatory protein. Eur J Biochem (1990) 1.43
The putative glucose 6-phosphate translocase gene is mutated in essentially all cases of glycogen storage disease type I non-a. Eur J Hum Genet (1999) 1.42
L: -2-Hydroxyglutaric aciduria, a disorder of metabolite repair. J Inherit Metab Dis (2008) 1.40
Heterogeneity in glucose sensitivity among pancreatic beta-cells is correlated to differences in glucose phosphorylation rather than glucose transport. EMBO J (1993) 1.34
Identification, cloning, and heterologous expression of a mammalian fructosamine-3-kinase. Diabetes (2000) 1.34
Formation of fructose 2,6-bisphosphate from fructose 1,6-bisphosphate by intramolecular cyclisation followed by alkaline hydrolysis. Eur J Biochem (1981) 1.31
Species and tissue distribution of the regulatory protein of glucokinase. Biochem J (1993) 1.30
The glucose sensor protein glucokinase is expressed in glucagon-producing alpha-cells. Proc Natl Acad Sci U S A (1996) 1.28
A gene on chromosome 11q23 coding for a putative glucose- 6-phosphate translocase is mutated in glycogen-storage disease types Ib and Ic. Am J Hum Genet (1998) 1.28
The stimulation of yeast phosphofructokinase by fructose 2,6-bisphosphate. FEBS Lett (1982) 1.27
Synthesis of a stimulator of phosphofructokinase, most likely fructose 2,6-bisphosphate, from phosphoric acid and fructose 6-phosphoric acid. Biochem Biophys Res Commun (1980) 1.26
Mutations in the glucokinase regulatory protein gene in 2p23 in obese French caucasians. Diabetologia (2003) 1.21
Phosphomannose isomerase deficiency: a carbohydrate-deficient glycoprotein syndrome with hepatic-intestinal presentation. Am J Hum Genet (1998) 1.20
RNAi screening in glioma stem-like cells identifies PFKFB4 as a key molecule important for cancer cell survival. Oncogene (2011) 1.20
Mechanistic studies of phosphoserine phosphatase, an enzyme related to P-type ATPases. J Biol Chem (1999) 1.17
Competitive inhibition of liver glucokinase by its regulatory protein. Eur J Biochem (1991) 1.16
Regulation of glucokinase by a fructose-1-phosphate-sensitive protein in pancreatic islets. Eur J Biochem (1990) 1.15
Inactivation of phosphofructokinase 2 by cyclic AMP - dependent protein kinase. Biochem Biophys Res Commun (1981) 1.12
Fructose 1-phosphate and the regulation of glucokinase activity in isolated hepatocytes. Eur J Biochem (1990) 1.12
Human L-3-phosphoserine phosphatase: sequence, expression and evidence for a phosphoenzyme intermediate. FEBS Lett (1997) 1.12
On the mechanism of inhibition of neutral liver fructose 1,6-bisphosphatase by fructose 2,6-bisphosphate. Eur J Biochem (1983) 1.11
The ability of adenosine to decrease the concentration of fructose 2,6-bisphosphate in isolated hepatocytes. A cyclic AMP-mediated effect. Biochem J (1984) 1.09
Kinetic properties and tissular distribution of mammalian phosphomannomutase isozymes. Biochem J (1999) 1.08
The mechanism by which glucose increases fructose 2,6-bisphosphate concentration in Saccharomyces cerevisiae. A cyclic-AMP-dependent activation of phosphofructokinase 2. Eur J Biochem (1984) 1.07
Effects of glucose and glucagon on the fructose 2,6-bisphosphate content of pancreatic islets and purified pancreatic B-cells. A comparison with isolated hepatocytes. Biochem J (1984) 1.07
High residual activity of PMM2 in patients' fibroblasts: possible pitfall in the diagnosis of CDG-Ia (phosphomannomutase deficiency). Am J Hum Genet (2001) 1.06
acs1 of Haemophilus influenzae type a capsulation locus region II encodes a bifunctional ribulose 5-phosphate reductase- CDP-ribitol pyrophosphorylase. J Bacteriol (1999) 1.06
Purification and properties of phosphofructokinase 2/fructose 2,6-bisphosphatase from chicken liver and from pigeon muscle. Eur J Biochem (1986) 1.06
The regulatory protein of glucokinase. Biochem Soc Trans (1997) 1.06
The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase. Biochimie (2005) 1.06
Amino acid conservation in animal glucokinases. Identification of residues implicated in the interaction with the regulatory protein. J Biol Chem (1996) 1.06
Effects of various metabolic conditions and of the trivalent arsenical melarsen oxide on the intracellular levels of fructose 2,6-bisphosphate and of glycolytic intermediates in Trypanosoma brucei. Eur J Biochem (1987) 1.05
Interference by pyruvate carboxylase in the measurement of acetyl-CoA carboxylase in crude liver preparations. Biochem J (1982) 1.05
Identification of the cDNA encoding human 6-phosphogluconolactonase, the enzyme catalyzing the second step of the pentose phosphate pathway(1). FEBS Lett (1999) 1.04
Purification and properties of spinach leaf phosphofructokinase 2/fructose 2,6-bisphosphatase. Eur J Biochem (1986) 1.04
Comparison of PMM1 with the phosphomannomutases expressed in rat liver and in human cells. FEBS Lett (1997) 1.03
Stimulation of glycolysis and accumulation of a stimulator of phosphofructokinase in hepatocytes incubated with vasopressin. Biochem J (1981) 1.02
Glucose-6-phosphatase mutation G188R confers an atypical glycogen storage disease type 1b phenotype. Pediatr Res (2000) 1.01
Binding of sorbitol 6-phosphate and of fructose 1-phosphate to the regulatory protein of liver glucokinase. Biochem J (1992) 1.01
Presence of a fructose-2,6-bisphosphate-insensitive pyrophosphate: fructose-6-phosphate phosphotransferase in the anaerobic protozoa Tritrichomonas foetus, Trichomonas vaginalis and Isotricha prostoma. Mol Biochem Parasitol (1989) 1.01
Effect of mutations on the sensitivity of human beta-cell glucokinase to liver regulatory protein. Diabetologia (1996) 1.01
Phosphomannomutase deficiency is the main cause of carbohydrate-deficient glycoprotein syndrome with type I isoelectrofocusing pattern of serum sialotransferrins. J Inherit Metab Dis (1997) 0.99
PMM (PMM1), the human homologue of SEC53 or yeast phosphomannomutase, is localized on chromosome 22q13. Genomics (1997) 0.99
Glucokinase regulatory protein is essential for the proper subcellular localisation of liver glucokinase. FEBS Lett (1999) 0.98
Phosphoserine phosphatase deficiency in a patient with Williams syndrome. J Med Genet (1997) 0.97
Effect of benzoate on the metabolism of fructose 2,6-bisphosphate in yeast. Eur J Biochem (1986) 0.97
Cloning and expression of a Xenopus liver cDNA encoding a fructose-phosphate-insensitive regulatory protein of glucokinase. Eur J Biochem (1994) 0.97
Study of the regulatory properties of glucokinase by site-directed mutagenesis: conversion of glucokinase to an enzyme with high affinity for glucose. Diabetes (2000) 0.96
The regulatory protein of glucokinase binds to the hepatocyte matrix, but, unlike glucokinase, does not translocate during substrate stimulation. Biochem J (1995) 0.96
Beneficial effects of L-serine and glycine in the management of seizures in 3-phosphoglycerate dehydrogenase deficiency. Ann Neurol (1998) 0.95
3-phosphoglycerate dehydrogenase deficiency in a patient with West syndrome. Dev Med Child Neurol (2000) 0.94
The stimulation of phosphofructokinase from human erythrocytes by fructose 2,6-bisphosphate. FEBS Lett (1982) 0.93
The mechanism by which ethanol decreases the concentration of fructose 2,6-bisphosphate in the liver. Biochem J (1984) 0.92
Carbohydrate-deficient glycoprotein syndrome type IA (phosphomannomutase-deficiency). Biochim Biophys Acta (1999) 0.92
Differential effects of glucose and fructose on liver L-type pyruvate kinase gene expression in vivo. J Biol Chem (1987) 0.92
Effect of ethylene treatment on the concentration of fructose-2,6-bisphosphate and on the activity of phosphofructokinase 2/fructose-2,6-bisphosphatase in banana. Eur J Biochem (1987) 0.91
Fructose 2,6-bisphosphate in yeast. Biochem Biophys Res Commun (1981) 0.91
Structure of the gene mutated in glycogen storage disease type Ib. Gene (1999) 0.89
Conversion of a synthetic fructosamine into its 3-phospho derivative in human erythrocytes. Biochem J (2000) 0.88
Variability in erythrocyte fructosamine 3-kinase activity in humans correlates with polymorphisms in the FN3K gene and impacts on haemoglobin glycation at specific sites. Diabetes Metab (2006) 0.86
Fructose 2,6-bisphosphate and germination of fungal spores. Proc Natl Acad Sci U S A (1983) 0.85
Fructosamine 3-kinase, an enzyme involved in protein deglycation. Biochem Soc Trans (2003) 0.85
Reaction of phosphofructokinase 2/fructose 2,6-bisphosphatase with monoclonal antibodies. A proof of the bifunctionality of the enzyme. Eur J Biochem (1986) 0.84
Pyrophosphate-dependent phosphofructokinase from the amoeba Naegleria fowleri, an AMP-sensitive enzyme. Biochem J (1993) 0.84
Conversion of fructose to glucose in the rabbit small intestine. A reappraisal of the direct pathway. Eur J Biochem (1993) 0.83
Role of cysteine in the dietary control of the expression of 3-phosphoglycerate dehydrogenase in rat liver. Biochem J (1999) 0.83
Towards a suggestive facial dysmorphism in adenylosuccinate lyase deficiency? J Med Genet (2002) 0.83
3-Phosphoglycerate dehydrogenase deficiency and 3-phosphoserine phosphatase deficiency: inborn errors of serine biosynthesis. J Inherit Metab Dis (1996) 0.83
Protein phosphorylation in the control of glycolysis and gluconeogenesis in the liver. Adv Cyclic Nucleotide Protein Phosphorylation Res (1984) 0.83
Fructose 2,6-bisphosphate hydrolyzing enzymes in higher plants. Plant Physiol (1989) 0.83
Demonstration of glycosomes (microbodies) in the Bodonid flagellate Trypanoplasma borelli (Protozoa, Kinetoplastida). Mol Biochem Parasitol (1988) 0.82
Investigation on the mechanism by which fructose, hexitols and other compounds regulate the translocation of glucokinase in rat hepatocytes. Biochem J (1997) 0.82