Property Summary

NCBI Gene PubMed Count 114
PubMed Score 245.31
PubTator Score 413.81

Knowledge Summary


No data available


  Differential Expression (13)

Disease log2 FC p
Multiple myeloma 1.041 5.3e-04
psoriasis -1.200 1.5e-03
osteosarcoma 1.349 4.1e-04
group 4 medulloblastoma -2.000 4.1e-06
glioblastoma -2.100 5.1e-04
atypical teratoid / rhabdoid tumor -1.700 1.5e-06
medulloblastoma, large-cell -1.500 4.6e-04
primitive neuroectodermal tumor -1.300 6.2e-05
pancreatic ductal adenocarcinoma liver m... -3.058 7.5e-04
adult high grade glioma -1.500 2.2e-04
subependymal giant cell astrocytoma -1.162 4.7e-02
ovarian cancer 1.700 4.5e-04
Down syndrome 1.300 4.0e-03

Gene RIF (50)

26241911 Study of the expression of the GDH1/2 in human steroidogenic organs revealed that, while GDH2 was expressed specifically in steroid-synthesizing cells, GDH1 was expressed both in the cells that produce steroids and in those that lack endocrine function.
25947346 GDH plays a critical role in colorectal cancer progression
25781533 Mutation in the GLUD1 gene is associated with hyperinsulinism/hyperammonemia.
25670081 argeting GDH1 by shRNA or a small molecule inhibitor R162 resulted in imbalanced redox homeostasis, leading to attenuated cancer cell proliferation and tumor growth.
25620628 side-chain interactions between 409 and 443 positions in the 'antenna' region of GDH are crucial for basal catalytic activity, allosteric regulation, and relative resistance to thermal inactivation
24771001 SIRT3 and glutamate dehydrogenase are altered in follicular cells of women with reduced ovarian reserve or advanced maternal age
24295216 The GDH1 is a key metabolic enzyme with emerging roles in insulin regulation. MitoNEET forms a covalent complex with GDH1 through disulfide bond formation and acts as an activator.
23636801 stromal expression of the glutamine-metabolism-related proteins GLS1, GDH, ASCT2 increases with worsening histological phyllodes tumor grade.
23575388 The data suggest a dual mechanism by which glutamate dehydrogenase activity modulates autophagy, i.e., by activating MTORC1 and by limiting the formation of reactive oxygen species.
23507704 HER2- type breast cancer had the highest expression of stromal GLS1, tumoral GDH, stromal GDH, and tumoral ASCT, while TNBC had the lowest tumoral GDH expression.
22924626 Lack of Glud1 in transgenic mouse brain inhibits GDH activity regardless of enzymatic direction, modified glutamate handling, decreased glutamate catabolism and increased glutamine levels without affecting synaptic transmission.
22658952 first N-terminal alpha helical structure is crucial for the mitochondrial import of hGDH2 and these findings may have implications in understanding the evolutionary mechanisms that led to the large mitochondrial targeting signals of human GDHs
22360420 A protein encoded by this locus was found to be differentially expressed in postmortem brains from patients with atypical frontotemporal lobar degeneration.
22106762 Report glutamate dehydrogenase mutations in Japanese patients with congenital hyperinsulinism and hyperammonemia syndrome.
21901531 GDH interactions with aristolochic acid induce apoptosis of renal tubular epithelial cells.
21813650 Green tea polyphenols control dysregulated glutamate dehydrogenase in transgenic mice by hijacking the ADP activation site.
21621574 Human GDH1 appears to act like bovine GDH1, but human GDH2 does not show the same enhancement of branched chain alpha-keto acid dehydrogenase complex enzyme activities.
21397652 Transgenic mice are useful in defining the molecular pathways affected by the over-activation of GLUD transgene in glutamatergic neurons of the brain and spinal cord.
21130127 [review] Hyperinsulinemic hypoglycemia and hyperammonemia demonstrate the important role of GDH in insulin regulation and illustrate unexpectedly important reasons for the unusually complex allosteric regulation of GDH.
20931523 In Chinese, R269H, S445L mutation of the GLUD1 leads to congenital hyperinsulinism.
20877624 Observational study of gene-disease association. (HuGE Navigator)
20659789 Observational study and genome-wide association study of gene-disease association. (HuGE Navigator)
20381070 This protein has been found differentially expressed in the anterior cingulate cortex in women patients with schizophrenia.
20201926 Observational study and genome-wide association study of gene-disease association. (HuGE Navigator)
20197649 Gradual decrease in leukocyte GLDH activity may be a key factor for neurodegenerative aging processes.
20164212 Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
19890003 Transgenic mice overexpressing glutamate dehydrogenase 1 exhibited decreases in long-term potentiation of synaptic activity and spine density in hippocampal neurons.
19826036 High glutamate dehydrogenase is associated with glioblastoma.
19690084 Heterozygous missense mutations were detected in 15 patients with HI/HA, 2 of which are novel (N410D and D451V).
19500843 down-regulation of GDH in response to decreased fetal liver output and reduced umbilical artery glutamate concentrations may occur in human fetal growth restriction pregnancies
19448744 Hence, while most of the hGDHs translocate into the mitochondria (a process associated with cleavage of the signal sequence), part of the protein localizes to the endoplasmic reticulum, probably serving additional functions
19156168 Clinical trial of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
19046187 Neurological disorders in HHS are more frequent than previously thought and might suggest that mutations in the GTP binding site of GDH could be associated with more frequent epilepsy.
19046183 Although GDH activity is also highly expressed in the brain, central nervous system (CNS) manifestations of GLUD1 activating mutations have remained relatively unexplored.
18688271 GLUD1 parental protein localizes to mitochondria and the cytoplasm, GLUD2 is specifically targeted to mitochondria.
18321734 we describe a family with a dominantly inherited mutation in GDH in Myoclonic absence epilepsy with photosensitivity.
16492972 Results describe the electrophysiological mechanisms underlying the dysregulated insulin secretion in pancreatic beta cells with glutamate dehydrogenase mutations.
16385451 Observational study of gene-disease association. (HuGE Navigator)
16151898 GLUD1 may have a role in hyperinsulinism/hyperammonaemia syndrome
15750346 results suggest that cysteine 323 plays an important role in catalysis by human GDH isozymes; C323 is not directly involved in allosteric regulation.
15378063 GLUD2 originated by retroposition from GLUD1 in the hominoid ancestor less than 23 million years ago.
15273247 inhibition of glutamate dehydrogenase results in sulfite neurotoxicity
15044002 These results suggest that the Ser443 residue plays an important role in the different thermal stability of human glutamate dehydrogenase isozymes (hGDH1 and hGDH2).
12324473 substitution of Ser for Arg-443 (but not substitution of Thr for Ser-331, Leu for Met-370, or Leu for Met-415) virtually abolished basal activity and totally abrogated the activation of the enzyme by l-leucine (1-10 mm) in the absence of other effectors
12193607 site-directed mutagenesis showing involvement of Glu(279)in NAD+ binding
12022886 Tyr187 is responsible for the efficient base binding of ADP to human GDH.
11950837 identify the structural basis for allosteric differences of GlUD1 and GLUD2
11903050 Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations
11872671 increased glutamate dehydrogenase activity confers on beta-cells the ability to secrete insulin in response to glutamine
11840195 Two missense mutations give rise to two different forms of congenital hyperinsulinism/hyperammonemia.

AA Sequence


Text Mined References (118)

PMID Year Title
26759084 2016 Early Presentation of Hyperinsulinism/Hyperammonemia Syndrome in Three Serbian Patients.
26758964 2016 Clinical and Genetic Characteristics, Management and Long-Term Follow-Up of Turkish Patients with Congenital Hyperinsulinism.
26740944 2015 Uncovering the molecular pathogenesis of congenital hyperinsulinism by panel gene sequencing in 32 Chinese patients.
26656609 2016 A novel mutation in the glutamate dehydrogenase (GLUD1) of a patient with congenital hyperinsulinism-hyperammonemia (HI/HA).
26511081 2016 Falsely Elevated Glucose Concentrations in Peritoneal Dialysis Patients Using Icodextrin.
26431509 2015 High Incidence of Heterozygous ABCC8 and HNF1A Mutations in Czech Patients With Congenital Hyperinsulinism.
26422656 2016 Protein profiles distinguish stable and progressive chronic lymphocytic leukemia.
26316429 2015 The Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia.
26268944 2015 Genotype and phenotype correlations in Iranian patients with hyperinsulinaemic hypoglycaemia.
26241911 2015 Expression of human GLUD1 and GLUD2 glutamate dehydrogenases in steroid producing tissues.
26045471 2015 Glutamine promotes ovarian cancer cell proliferation through the mTOR/S6 pathway.
25972930 2015 Clinical and molecular data from 61 Brazilian cases of Congenital Hyperinsulinemic Hypoglycemia.
25947346 2015 Glutamate dehydrogenase is a novel prognostic marker and predicts metastases in colorectal cancer patients.
25944712 2015 N-terminome analysis of the human mitochondrial proteome.
25803269 2015 Maternal Smoking Dysregulates Protein Expression in Second Trimester Human Fetal Livers in a Sex-Specific Manner.
25781533 2015 Hyperinsulinemic hypoglycemia: think of hyperinsulinism/hyperammonemia (HI/HA) syndrome caused by mutations in the GLUD1 gene.
25780012 2015 Expression of metabolism-related proteins in lacrimal gland adenoid cystic carcinoma.
25733449 2015 Molecular mechanisms of congenital hyperinsulinism.
25719198 2015 Metabolic phenotypes in pancreatic cancer.
25712828 2015 A case with neonatal hyperinsulinemic hypoglycemia: It is a characteristic complication of Sotos syndrome.
25700560 2015 SIRT5 regulation of ammonia-induced autophagy and mitophagy.
25670081 2015 Glutamate dehydrogenase 1 signals through antioxidant glutathione peroxidase 1 to regulate redox homeostasis and tumor growth.
25620628 2015 Side-chain interactions in the regulatory domain of human glutamate dehydrogenase determine basal activity and regulation.
25538565 2014 GABA-A and NMDA receptor subunit mRNA expression is altered in the caudate but not the putamen of the postmortem brains of alcoholics.
25496516 2014 Site-specific metabolic phenotypes in metastatic breast cancer.
25458842 2014 Glutamine oxidation maintains the TCA cycle and cell survival during impaired mitochondrial pyruvate transport.
25414017 2014 Interaction of islet ?-cell and ?-cell in the regulation of glucose homeostasis in HI/HA syndrome patients with the GDH(H454Y) mutation.
25370487 2015 Localization of SUCLA2 and SUCLG2 subunits of succinyl CoA ligase within the cerebral cortex suggests the absence of matrix substrate-level phosphorylation in glial cells of the human brain.
25317244 2014 Molecular mechanisms of protein induced hyperinsulinaemic hypoglycaemia.
25117148 2014 Novel ABCC8 (SUR1) gene mutations in Asian Indian children with congenital hyperinsulinemic hypoglycemia.
25077087 2014 Congenital hyperinsulinism: current status and future perspectives.
25053597 2014 Expression of metabolism-related proteins in invasive lobular carcinoma: comparison to invasive ductal carcinoma.
25024374 2014 Mitochondrial GTP insensitivity contributes to hypoglycemia in hyperinsulinemia hyperammonemia by inhibiting glucagon release.
25008049 2014 Mutational analysis of ABCC8, KCNJ11, GLUD1, HNF4A and GCK genes in 30 Chinese patients with congenital hyperinsulinism.
24771001 2014 Mitochondrial SIRT3 and its target glutamate dehydrogenase are altered in follicular cells of women with reduced ovarian reserve or advanced maternal age.
24295216 2013 Identification of disulfide bond formation between MitoNEET and glutamate dehydrogenase 1.
24275569 2014 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
23636801 2013 The expression of glutamine-metabolism-related proteins in breast phyllodes tumors.
23575388 2013 Glutamate dehydrogenase contributes to leucine sensing in the regulation of autophagy.
23507704 2013 Expression of glutamine metabolism-related proteins according to molecular subtype of breast cancer.
22926577 2012 Quantitative proteomic analysis of human substantia nigra in Alzheimer's disease, Huntington's disease and Multiple sclerosis.
22924626 2012 Deletion of glutamate dehydrogenase 1 (Glud1) in the central nervous system affects glutamate handling without altering synaptic transmission.
22658952 2012 The complex regulation of human glud1 and glud2 glutamate dehydrogenases and its implications in nerve tissue biology.
22360420 2012 Proteomic analysis identifies dysfunction in cellular transport, energy, and protein metabolism in different brain regions of atypical frontotemporal lobar degeneration.
22106762 2011 Spectrum of glutamate dehydrogenase mutations in Japanese patients with congenital hyperinsulinism and hyperammonemia syndrome.
21901531 2011 Glutamate dehydrogenase requirement for apoptosis induced by aristolochic acid in renal tubular epithelial cells.
21813650 2011 Green tea polyphenols control dysregulated glutamate dehydrogenase in transgenic mice by hijacking the ADP activation site.
21621574 2011 Interaction between glutamate dehydrogenase (GDH) and L-leucine catabolic enzymes: intersecting metabolic pathways.
21397652 2011 Neuronal Glud1 (glutamate dehydrogenase 1) over-expressing mice: increased glutamate formation and synaptic release, loss of synaptic activity, and adaptive changes in genomic expression.
21269460 2011 Initial characterization of the human central proteome.
21130127 2011 Two genetic forms of hyperinsulinemic hypoglycemia caused by dysregulation of glutamate dehydrogenase.
20931523 2010 [Mutation analysis of the GLUD1 gene in patients with glutamate dehydrogenase congenital hyperinsulinism].
20877624 2010 Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
20736409 2010 Systematic mapping and functional analysis of a family of human epididymal secretory sperm-located proteins.
20659789 2010 Common variants conferring risk of schizophrenia: a pathway analysis of GWAS data.
20381070 2010 Sex-specific proteome differences in the anterior cingulate cortex of schizophrenia.
20201926 2010 Human variation in alcohol response is influenced by variation in neuronal signaling genes.
20197649 2010 Glutamate dehydrogenase activity in leukocytes and ageing.
20164212 2010 Diazoxide-responsive hyperinsulinemic hypoglycemia caused by HNF4A gene mutations.
19890003 2009 Transgenic expression of Glud1 (glutamate dehydrogenase 1) in neurons: in vivo model of enhanced glutamate release, altered synaptic plasticity, and selective neuronal vulnerability.
19826036 2009 Glioblastoma cells require glutamate dehydrogenase to survive impairments of glucose metabolism or Akt signaling.
19690084 2009 Hyperinsulinism-hyperammonaemia syndrome: novel mutations in the GLUD1 gene and genotype-phenotype correlations.
19608861 2009 Lysine acetylation targets protein complexes and co-regulates major cellular functions.
19500843 2009 Expression of enzymes regulating placental ammonia homeostasis in human fetal growth restricted pregnancies.
19448744 2009 Human GLUD1 and GLUD2 glutamate dehydrogenase localize to mitochondria and endoplasmic reticulum.
19156168 2009 Pharmacogenetics of antipsychotic response in the CATIE trial: a candidate gene analysis.
19046187 2008 Neurological aspects of hyperinsulinism-hyperammonaemia syndrome.
19046183 2008 Neurological aspects in hyperinsulinism-hyperammonaemia syndrome.
18928469 2008 Hyperinsulinism and hyperammonaemia syndrome due to a novel missense mutation in the allosteric domain of the glutamate dehydrogenase 1 gene.
18688271 2008 Mitochondrial targeting adaptation of the hominoid-specific glutamate dehydrogenase driven by positive Darwinian selection.
18321734 2008 Myoclonic absence epilepsy with photosensitivity and a gain of function mutation in glutamate dehydrogenase.
16959573 2006 SIRT4 inhibits glutamate dehydrogenase and opposes the effects of calorie restriction in pancreatic beta cells.
16492972 2006 Unregulated insulin secretion by pancreatic beta cells in hyperinsulinism/hyperammonemia syndrome: role of glutamate dehydrogenase, ATP-sensitive potassium channel, and nonselective cation channel.
16385451 2006 A scan of chromosome 10 identifies a novel locus showing strong association with late-onset Alzheimer disease.
16344560 2006 Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes.
16151898 2005 A case of hyperinsulinism/hyperammonaemia syndrome with reduced carbamoyl-phosphate synthetase-1 activity in liver: a pitfall in enzymatic diagnosis for hyperammonaemia.
16023112 2005 Identification of ADP-ribosylation site in human glutamate dehydrogenase isozymes.
15750346 2005 Critical role of the cysteine 323 residue in the catalytic activity of human glutamate dehydrogenase isozymes.
15609325 2005 Identification and validation of novel ERBB2 (HER2, NEU) targets including genes involved in angiogenesis.
15578726 2005 Molecular basis of human glutamate dehydrogenase regulation under changing energy demands.
15489334 2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15378063 2004 Birth and adaptive evolution of a hominoid gene that supports high neurotransmitter flux.
15273247 2004 A mechanism of sulfite neurotoxicity: direct inhibition of glutamate dehydrogenase.
15164054 2004 The DNA sequence and comparative analysis of human chromosome 10.
15044002 2004 Important role of Ser443 in different thermal stability of human glutamate dehydrogenase isozymes.
14760703 2004 Proteomic identification of brain proteins that interact with dynein light chain LC8.
14702039 2004 Complete sequencing and characterization of 21,243 full-length human cDNAs.
12742085 Study of structure-function relationships in human glutamate dehydrogenases reveals novel molecular mechanisms for the regulation of the nerve tissue-specific (GLUD2) isoenzyme.
12653548 2003 Structural studies on ADP activation of mammalian glutamate dehydrogenase and the evolution of regulation.
12477932 2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.
12324473 2002 Substitution of Ser for Arg-443 in the regulatory domain of human housekeeping (GLUD1) glutamate dehydrogenase virtually abolishes basal activity and markedly alters the activation of the enzyme by ADP and L-leucine.
12193607 2002 Importance of glutamate 279 for the coenzyme binding of human glutamate dehydrogenase.
12107413 2002 Expressed sequence tag analysis of adult human lens for the NEIBank Project: over 2000 non-redundant transcripts, novel genes and splice variants.
12054821 2002 The structure of apo human glutamate dehydrogenase details subunit communication and allostery.
11950837 2002 Single amino acid substitution (G456A) in the vicinity of the GTP binding domain of human housekeeping glutamate dehydrogenase markedly attenuates GTP inhibition and abolishes the cooperative behavior of the enzyme.
11903050 2002 Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations.
11502802 2001 Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome.
11297618 2001 Hyperinsulinism/hyperammonemia syndrome in children with regulatory mutations in the inhibitory guanosine triphosphate-binding domain of glutamate dehydrogenase.
11254391 2001 Structures of bovine glutamate dehydrogenase complexes elucidate the mechanism of purine regulation.
11214910 2001 Novel missense mutations outside the allosteric domain of glutamate dehydrogenase are prevalent in European patients with the congenital hyperinsulinism-hyperammonemia syndrome.
11032875 2000 Nerve tissue-specific (GLUD2) and housekeeping (GLUD1) human glutamate dehydrogenases are regulated by distinct allosteric mechanisms: implications for biologic function.
10636977 2000 Novel missense mutations in the glutamate dehydrogenase gene in the congenital hyperinsulinism-hyperammonemia syndrome.
10338089 1999 Congenital hyperinsulinism: molecular basis of a heterogeneous disease.
9571255 1998 Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
8486350 1993 The human glutamate dehydrogenase gene family: gene organization and structural characterization.
8314555 1993 Structure and expression analysis of a member of the human glutamate dehydrogenase (GLUD) gene family mapped to chromosome 10p11.2.
8244384 1993 Three human glutamate dehydrogenase genes (GLUD1, GLUDP2, and GLUDP3) are located on chromosome 10q, but are not closely physically linked.
8125298 1994 Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides.
6121377 1982 Abnormal glutamate metabolism in an adult-onset degenerative neurological disorder.
3997814 1985 Regulation of aminotransferase-glutamate dehydrogenase interactions by carbamyl phosphate synthase-I, Mg2+ plus leucine versus citrate and malate.
3585334 1987 Isolation of a human brain cDNA for glutamate dehydrogenase.
3426581 1987 Comparison of human brain and liver glutamate dehydrogenase cDNAS.
3399399 1988 Complete nucleotide sequence of human glutamate dehydrogenase cDNA.
3377777 1988 Molecular cloning and nucleotide sequence of the cDNA for human liver glutamate dehydrogenase precursor.
3368458 1988 Isolation and characterization of cDNA clones encoding human liver glutamate dehydrogenase: evidence for a small gene family.
2757358 1989 Assignment of the GDH loci to human chromosomes 10q23 and Xq24 by in situ hybridization.
1286669 1992 Human liver protein map: a reference database established by microsequencing and gel comparison.
429360 1979 Partial amino acid sequence of the glutamate dehydrogenase of human liver and a revision of the sequence of the bovine enzyme.