Published in J Biol Chem on November 19, 2002
Interplay between lipids and branched-chain amino acids in development of insulin resistance. Cell Metab (2012) 3.26
The MODY1 gene HNF-4alpha regulates selected genes involved in insulin secretion. J Clin Invest (2005) 1.78
Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. J Biol Chem (2010) 1.68
Anaphase-promoting complex/cyclosome-Cdh1 coordinates glycolysis and glutaminolysis with transition to S phase in human T lymphocytes. Proc Natl Acad Sci U S A (2010) 1.50
The dynamin-related GTPase Opa1 is required for glucose-stimulated ATP production in pancreatic beta cells. Mol Biol Cell (2011) 1.26
Novel inhibitors complexed with glutamate dehydrogenase: allosteric regulation by control of protein dynamics. J Biol Chem (2009) 1.17
Chronic leucine supplementation improves glycemic control in etiologically distinct mouse models of obesity and diabetes mellitus. Nutr Metab (Lond) (2010) 1.15
Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt. J Biol Chem (2008) 1.10
Glucokinase activation repairs defective bioenergetics of islets of Langerhans isolated from type 2 diabetics. Am J Physiol Endocrinol Metab (2011) 1.03
The structure and allosteric regulation of mammalian glutamate dehydrogenase. Arch Biochem Biophys (2011) 1.03
The mitochondrial isoform of phosphoenolpyruvate carboxykinase (PEPCK-M) and glucose homeostasis: has it been overlooked? Biochim Biophys Acta (2013) 1.02
Higher branched-chain amino acid intake is associated with a lower prevalence of being overweight or obese in middle-aged East Asian and Western adults. J Nutr (2010) 1.01
Transamination is required for {alpha}-ketoisocaproate but not leucine to stimulate insulin secretion. J Biol Chem (2010) 1.01
Regulation of glucagon secretion in normal and diabetic human islets by γ-hydroxybutyrate and glycine. J Biol Chem (2012) 1.00
Studies with leucine, beta-hydroxybutyrate and ATP citrate lyase-deficient beta cells support the acetoacetate pathway of insulin secretion. Biochim Biophys Acta (2008) 1.00
Dose- and Glucose-Dependent Effects of Amino Acids on Insulin Secretion from Isolated Mouse Islets and Clonal INS-1E Beta-Cells. Rev Diabet Stud (2009) 0.99
Green tea polyphenols control dysregulated glutamate dehydrogenase in transgenic mice by hijacking the ADP activation site. J Biol Chem (2011) 0.98
Mitochondrial GTP insensitivity contributes to hypoglycemia in hyperinsulinemia hyperammonemia by inhibiting glucagon release. Diabetes (2014) 0.93
SIRT4 Is a Lysine Deacylase that Controls Leucine Metabolism and Insulin Secretion. Cell Metab (2017) 0.92
Hyperinsulinemic Hypoglycemia - The Molecular Mechanisms. Front Endocrinol (Lausanne) (2016) 0.91
The SLC38 family of sodium-amino acid co-transporters. Pflugers Arch (2013) 0.91
High throughput screening reveals several new classes of glutamate dehydrogenase inhibitors. Biochemistry (2007) 0.90
Conversion into GABA (gamma-aminobutyric acid) may reduce the capacity of L-glutamine as an insulin secretagogue. Biochem J (2004) 0.88
Delineation of glutamate pathways and secretory responses in pancreatic islets with β-cell-specific abrogation of the glutamate dehydrogenase. Mol Biol Cell (2012) 0.87
Segregation of two glutaminase isoforms in islets of Langerhans. Biochem J (2004) 0.86
The structure and allosteric regulation of glutamate dehydrogenase. Neurochem Int (2010) 0.85
Bisphenol a exposure disrupts metabolic health across multiple generations in the mouse. Endocrinology (2015) 0.84
Targeted disruption of pancreatic-derived factor (PANDER, FAM3B) impairs pancreatic beta-cell function. Diabetes (2010) 0.83
Glucose-stimulated single pancreatic islets sustain increased cytosolic ATP levels during initial Ca2+ influx and subsequent Ca2+ oscillations. J Biol Chem (2013) 0.82
Accumulation of 3-hydroxytetradecenoic acid: Cause or corollary of glucolipotoxic impairment of pancreatic β-cell bioenergetics? Mol Metab (2015) 0.82
Mechanisms of amino acid-stimulated insulin secretion in congenital hyperinsulinism. Acta Biochim Biophys Sin (Shanghai) (2012) 0.80
Increased fetal insulin concentrations for one week fail to improve insulin secretion or β-cell mass in fetal sheep with chronically reduced glucose supply. Am J Physiol Regul Integr Comp Physiol (2012) 0.79
α/β-Hydrolase domain-6 and saturated long chain monoacylglycerol regulate insulin secretion promoted by both fuel and non-fuel stimuli. Mol Metab (2015) 0.79
The Ratio of Dietary Branched-Chain Amino Acids is Associated with a Lower Prevalence of Obesity in Young Northern Chinese Adults: An Internet-Based Cross-Sectional Study. Nutrients (2015) 0.78
Acute exposure to a precursor of advanced glycation end products induces a dual effect on the rat pancreatic islet function. Int J Endocrinol (2014) 0.78
Mitochondrial regulation of β-cell function: maintaining the momentum for insulin release. Mol Aspects Med (2015) 0.77
The association of DNA methylation and brain volume in healthy individuals and schizophrenia patients. Schizophr Res (2015) 0.77
Plasma Metabolomic Changes following PI3K Inhibition as Pharmacodynamic Biomarkers: Preclinical Discovery to Phase I Trial Evaluation. Mol Cancer Ther (2016) 0.76
Novel metabolic and physiological functions of branched chain amino acids: a review. J Anim Sci Biotechnol (2017) 0.75
Insulin and SGK1 reduce the function of Na+/monocarboxylate transporter 1 (SMCT1/SLC5A8). Am J Physiol Cell Physiol (2016) 0.75
Extreme obesity induces massive beta cell expansion in mice through self-renewal and does not alter the beta cell lineage. Diabetologia (2016) 0.75
Branched-chain amino acid supplementation restores reduced insulinotropic activity of a low-protein diet through the vagus nerve in rats. Nutr Metab (Lond) (2017) 0.75
Functional and Metabolomic Consequences of ATP-dependent Potassium Channel Inactivation in Human Islets. Diabetes (2017) 0.75
Beyond aerobic glycolysis: transformed cells can engage in glutamine metabolism that exceeds the requirement for protein and nucleotide synthesis. Proc Natl Acad Sci U S A (2007) 15.88
Myc regulates a transcriptional program that stimulates mitochondrial glutaminolysis and leads to glutamine addiction. Proc Natl Acad Sci U S A (2008) 11.71
A genome-wide association study identifies KIAA0350 as a type 1 diabetes gene. Nature (2007) 6.10
Allosteric activators of glucokinase: potential role in diabetes therapy. Science (2003) 3.97
A novel susceptibility locus for type 1 diabetes on Chr12q13 identified by a genome-wide association study. Diabetes (2008) 2.90
Cross-sectional multicenter study of patients with urea cycle disorders in the United States. Mol Genet Metab (2008) 2.90
Multiple phenotypes in phosphoglucomutase 1 deficiency. N Engl J Med (2014) 2.62
Structural and functional abnormalities in the islets isolated from type 2 diabetic subjects. Diabetes (2004) 2.22
Establishing a consortium for the study of rare diseases: The Urea Cycle Disorders Consortium. Mol Genet Metab (2010) 2.07
Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab (2007) 2.03
Metabolic signaling in fuel-induced insulin secretion. Cell Metab (2013) 2.00
A pilot study of in vivo liver-directed gene transfer with an adenoviral vector in partial ornithine transcarbamylase deficiency. Hum Gene Ther (2002) 1.96
Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan. J Pediatr (2007) 1.92
Comparative genetic analysis of inflammatory bowel disease and type 1 diabetes implicates multiple loci with opposite effects. Hum Mol Genet (2010) 1.81
The MODY1 gene HNF-4alpha regulates selected genes involved in insulin secretion. J Clin Invest (2005) 1.78
Very long-chain acyl-CoA dehydrogenase deficiency in a patient with normal newborn screening by tandem mass spectrometry. J Pediatr (2010) 1.75
Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. J Biol Chem (2010) 1.68
Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations. J Clin Invest (2008) 1.66
Primary coenzyme Q deficiency in Pdss2 mutant mice causes isolated renal disease. PLoS Genet (2008) 1.66
Foxa2 controls vesicle docking and insulin secretion in mature Beta cells. Cell Metab (2007) 1.66
Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase. J Biol Chem (2006) 1.64
Foxa2 controls Pdx1 gene expression in pancreatic beta-cells in vivo. Diabetes (2002) 1.60
Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations. J Pediatr (2006) 1.57
Foxa2 regulates multiple pathways of insulin secretion. J Clin Invest (2004) 1.57
Follow-up analysis of genome-wide association data identifies novel loci for type 1 diabetes. Diabetes (2008) 1.56
Foxa1 and Foxa2 maintain the metabolic and secretory features of the mature beta-cell. Mol Endocrinol (2010) 1.47
Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue. Diabetes (2007) 1.45
N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers. Pediatr Res (2008) 1.44
Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. Clin Chem (2007) 1.37
The second activating glucokinase mutation (A456V): implications for glucose homeostasis and diabetes therapy. Diabetes (2002) 1.35
A signaling role of glutamine in insulin secretion. J Biol Chem (2004) 1.33
Insights into the biochemical and genetic basis of glucokinase activation from naturally occurring hypoglycemia mutations. Diabetes (2003) 1.33
Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism. J Pediatr (2006) 1.29
The ketogenic diet and brain metabolism of amino acids: relationship to the anticonvulsant effect. Annu Rev Nutr (2007) 1.28
The structure of apo human glutamate dehydrogenase details subunit communication and allostery. J Mol Biol (2002) 1.27
Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. J Clin Endocrinol Metab (2007) 1.25
Rapamycin has a deleterious effect on MIN-6 cells and rat and human islets. Diabetes (2003) 1.25
Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations. Diabetes (2009) 1.23
Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents. Mol Endocrinol (2006) 1.21
From clinicogenetic studies of maturity-onset diabetes of the young to unraveling complex mechanisms of glucokinase regulation. Diabetes (2006) 1.20
Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism. Pediatr Diabetes (2009) 1.20
Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. J Pediatr (2004) 1.18
N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. Pediatrics (2010) 1.17
Familial leucine-sensitive hypoglycemia of infancy due to a dominant mutation of the beta-cell sulfonylurea receptor. J Clin Endocrinol Metab (2004) 1.16
A novel KCNJ11 mutation associated with congenital hyperinsulinism reduces the intrinsic open probability of beta-cell ATP-sensitive potassium channels. J Biol Chem (2005) 1.16
Permanent neonatal diabetes caused by glucokinase deficiency: inborn error of the glucose-insulin signaling pathway. Diabetes (2003) 1.15
Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice. J Biol Chem (2006) 1.15
Brain amino acid requirements and toxicity: the example of leucine. J Nutr (2005) 1.14
Untangling the glutamate dehydrogenase allosteric nightmare. Trends Biochem Sci (2008) 1.13
Insights into the structure and regulation of glucokinase from a novel mutation (V62M), which causes maturity-onset diabetes of the young. J Biol Chem (2005) 1.11
Diazoxide-unresponsive congenital hyperinsulinism in children with dominant mutations of the β-cell sulfonylurea receptor SUR1. Diabetes (2011) 1.10
Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt. J Biol Chem (2008) 1.10
Structural studies on ADP activation of mammalian glutamate dehydrogenase and the evolution of regulation. Biochemistry (2003) 1.10
Clinical and molecular characterization of a dominant form of congenital hyperinsulinism caused by a mutation in the high-affinity sulfonylurea receptor. Diabetes (2003) 1.09
Novel presentations of congenital hyperinsulinism due to mutations in the MODY genes: HNF1A and HNF4A. J Clin Endocrinol Metab (2012) 1.09
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis. Mol Genet Metab (2009) 1.09
A mutation in the TMD0-L0 region of sulfonylurea receptor-1 (L225P) causes permanent neonatal diabetes mellitus (PNDM). Diabetes (2007) 1.09
Newborn screening by tandem mass spectrometry for medium-chain Acyl-CoA dehydrogenase deficiency: a cost-effectiveness analysis. Pediatrics (2003) 1.09
Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations. Biochem J (2002) 1.08
Response of brain amino acid metabolism to ketosis. Neurochem Int (2005) 1.08
Inflammation-induced preterm birth alters neuronal morphology in the mouse fetal brain. J Neurosci Res (2010) 1.06
The hyperinsulinism/hyperammonemia syndrome. Rev Endocr Metab Disord (2010) 1.06
Effects of a glucokinase activator on hepatic intermediary metabolism: study with 13C-isotopomer-based metabolomics. Biochem J (2012) 1.06
Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes. J Clin Endocrinol Metab (2004) 1.05
Advances in diagnosis and treatment of hyperinsulinism in infants and children. J Clin Endocrinol Metab (2002) 1.04
Glucokinase activation repairs defective bioenergetics of islets of Langerhans isolated from type 2 diabetics. Am J Physiol Endocrinol Metab (2011) 1.03
Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion. J Biol Chem (2008) 1.03
The structure and allosteric regulation of mammalian glutamate dehydrogenase. Arch Biochem Biophys (2011) 1.03