Published in Trends Biochem Sci on September 24, 2008
Glioblastoma cells require glutamate dehydrogenase to survive impairments of glucose metabolism or Akt signaling. Cancer Res (2009) 3.15
Novel inhibitors complexed with glutamate dehydrogenase: allosteric regulation by control of protein dynamics. J Biol Chem (2009) 1.17
Leucine metabolism in regulation of insulin secretion from pancreatic beta cells. Nutr Rev (2010) 1.03
Binding leverage as a molecular basis for allosteric regulation. PLoS Comput Biol (2011) 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
Atomic structure of salutaridine reductase from the opium poppy (Papaver somniferum). J Biol Chem (2010) 0.91
Global characterization of in vivo enzyme catalytic rates and their correspondence to in vitro kcat measurements. Proc Natl Acad Sci U S A (2016) 0.89
The structure and allosteric regulation of glutamate dehydrogenase. Neurochem Int (2010) 0.85
Structural basis for leucine-induced allosteric activation of glutamate dehydrogenase. J Biol Chem (2011) 0.85
Glutamate dehydrogenase in brain mitochondria: do lipid modifications and transient metabolon formation influence enzyme activity? Neurochem Int (2011) 0.81
Glutamate dehydrogenase 1 and SIRT4 regulate glial development. Glia (2012) 0.80
An enzymatic bridge between carbohydrate and amino acid metabolism: regulation of glutamate dehydrogenase by reversible phosphorylation in a severe hypoxia-tolerant crayfish. J Comp Physiol B (2011) 0.80
Two genetic forms of hyperinsulinemic hypoglycemia caused by dysregulation of glutamate dehydrogenase. Neurochem Int (2010) 0.79
Glutaminolysis as a target for cancer therapy. Oncogene (2015) 0.78
Determination of glutamate dehydrogenase activity and its kinetics in mouse tissues using metabolic mapping (quantitative enzyme histochemistry). J Histochem Cytochem (2014) 0.76
Replication of the Shrimp Virus WSSV Depends on Glutamate-Driven Anaplerosis. PLoS One (2016) 0.75
A novel mechanism of V-type zinc inhibition of glutamate dehydrogenase results from disruption of subunit interactions necessary for efficient catalysis. FEBS J (2011) 0.75
The Glutamate Dehydrogenase Pathway and Its Roles in Cell and Tissue Biology in Health and Disease. Biology (Basel) (2017) 0.75
A severe case of hyperinsulinism due to hemizygous activating mutation of glutamate dehydrogenase. Pediatr Diabetes (2017) 0.75
A genome-wide association study identifies KIAA0350 as a type 1 diabetes gene. Nature (2007) 6.10
A novel susceptibility locus for type 1 diabetes on Chr12q13 identified by a genome-wide association study. Diabetes (2008) 2.90
Multiple phenotypes in phosphoglucomutase 1 deficiency. N Engl J Med (2014) 2.62
Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab (2007) 2.03
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
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
Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase. J Biol Chem (2006) 1.64
Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations. J Pediatr (2006) 1.57
Follow-up analysis of genome-wide association data identifies novel loci for type 1 diabetes. Diabetes (2008) 1.56
Regulation of leucine-stimulated insulin secretion and glutamine metabolism in isolated rat islets. J Biol Chem (2002) 1.53
Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue. Diabetes (2007) 1.45
Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. Clin Chem (2007) 1.37
A signaling role of glutamine in insulin secretion. J Biol Chem (2004) 1.33
Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism. J Pediatr (2006) 1.29
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
Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations. Diabetes (2009) 1.23
Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism. Pediatr Diabetes (2009) 1.20
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
Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice. J Biol Chem (2006) 1.15
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
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
The hyperinsulinism/hyperammonemia syndrome. Rev Endocr Metab Disord (2010) 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
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
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
Destabilization of ATP-sensitive potassium channel activity by novel KCNJ11 mutations identified in congenital hyperinsulinism. J Biol Chem (2008) 1.03
Sulfonylurea receptor 1 mutations that cause opposite insulin secretion defects with chemical chaperone exposure. J Biol Chem (2009) 1.01
Regulation of glucagon secretion in normal and diabetic human islets by γ-hydroxybutyrate and glycine. J Biol Chem (2012) 1.00
Evolution of glutamate dehydrogenase regulation of insulin homeostasis is an example of molecular exaptation. Biochemistry (2004) 0.99
Central nervous system hyperexcitability associated with glutamate dehydrogenase gain of function mutations. J Pediatr (2005) 0.99
Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism. Mod Pathol (2006) 0.98
Green tea polyphenols control dysregulated glutamate dehydrogenase in transgenic mice by hijacking the ADP activation site. J Biol Chem (2011) 0.98
The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography. J Clin Endocrinol Metab (2006) 0.97
GLP-1 receptor antagonist exendin-(9-39) elevates fasting blood glucose levels in congenital hyperinsulinism owing to inactivating mutations in the ATP-sensitive K+ channel. Diabetes (2012) 0.96
The surgical management of insulinomas in children. J Pediatr Surg (2013) 0.94
Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry: acyl-CoA profiles in short-chain fatty acid oxidation defects. Mol Genet Metab (2012) 0.93
Accuracy of PET/CT Scan in the diagnosis of the focal form of congenital hyperinsulinism. J Pediatr Surg (2013) 0.90
Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism. J Pediatr Surg (2007) 0.89
Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism. J Pediatr Surg (2012) 0.88
Determination of insulin for the diagnosis of hyperinsulinemic hypoglycemia. Best Pract Res Clin Endocrinol Metab (2013) 0.88
Hepatocyte nuclear factor 4α gene mutation associated with familial neonatal hyperinsulinism and maturity-onset diabetes of the young. J Pediatr (2011) 0.87
Dominant form of congenital hyperinsulinism maps to HK1 region on 10q. Horm Res Paediatr (2013) 0.87
Histopathology of congenital hyperinsulinism: retrospective study with genotype correlations. Pediatr Dev Pathol (2003) 0.87
Pancreatic surgery in infants with Beckwith-Wiedemann syndrome and hyperinsulinism. J Pediatr Surg (2013) 0.87
A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy. J Pediatr Surg (2004) 0.86
The value of radiologic interventions and (18)F-DOPA PET in diagnosing and localizing focal congenital hyperinsulinism: systematic review and meta-analysis. Mol Imaging Biol (2013) 0.86
A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism. Semin Pediatr Surg (2011) 0.86
Prenatal diagnosis and postnatal management of diffuse congenital hyperinsulinism: a case report. Fetal Diagn Ther (2006) 0.86
Rare forms of congenital hyperinsulinism. Semin Pediatr Surg (2011) 0.86
The structure and allosteric regulation of glutamate dehydrogenase. Neurochem Int (2010) 0.85
Glutaminolysis and insulin secretion: from bedside to bench and back. Diabetes (2002) 0.85
Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism. Arch Dis Child Fetal Neonatal Ed (2013) 0.85
Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism. J Pediatr Nurs (2005) 0.85
Poor specificity of low growth hormone and cortisol levels during fasting hypoglycemia for the diagnoses of growth hormone deficiency and adrenal insufficiency. Pediatrics (2008) 0.84
Glutamate dehydrogenase: structure, allosteric regulation, and role in insulin homeostasis. Neurochem Res (2013) 0.84
Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy. Am J Surg Pathol (2004) 0.83
Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI). Pediatr Endocrinol Rev (2004) 0.83
Intussusception after pancreatic surgery in children: a case series. J Pediatr Surg (2010) 0.82
Nesidioblastosis no longer! It's all about genetics. J Clin Endocrinol Metab (2011) 0.80
Characterization and functional restoration of a potassium channel Kir6.2 pore mutation identified in congenital hyperinsulinism. J Biol Chem (2009) 0.78
Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways. PLoS One (2012) 0.78
Pregnancy in glycogen storage disease type Ib: gestational care and report of first successful deliveries. J Inherit Metab Dis (2010) 0.76
Neurological aspects in hyperinsulinism-hyperammonaemia syndrome. Dev Med Child Neurol (2008) 0.75
Research initiatives in neonatal hypoglycemia. J Pediatr (2010) 0.75
Blood glucose control during selective arterial stimulation and venous sampling for localization of focal hyperinsulinism lesions in anesthetized children. Anesth Analg (2004) 0.75