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GlutathioneGlutathione TransferaseGlutathione PeroxidaseGlutathione ReductaseGlutathione DisulfideGlutathione SynthaseEnergy MetabolismLipid MetabolismButhionine SulfoximineGlutathione S-Transferase piLiverOxidation-ReductionGlutamate-Cysteine LigaseOxidative StressDinitrochlorobenzeneAntioxidantsMethionine SulfoximineMetabolismSulfhydryl CompoundsGlucoseKineticsBiotransformationSeleniumCatalaseChromatography, High Pressure LiquidMaleatesIsoenzymesCysteineMicrosomes, LiverMolecular Sequence DataLipid PeroxidationSuperoxide DismutaseAcetylcysteinegamma-GlutamyltransferaseCytochrome P-450 Enzyme SystemHydrogen PeroxideReactive Oxygen SpeciesMetabolic Detoxication, DrugAmino Acid SequenceCystineRats, Sprague-DawleyCells, CulturedRats, Inbred StrainsTime FactorsGlycolysisMetabolic Networks and PathwaysErythrocytesRNA, MessengerMalondialdehydeRats, WistarSubstrate SpecificityDose-Response Relationship, DrugNADPModels, BiologicalMitochondriaOxygen ConsumptionOxidoreductasesBiological TransportFatty AcidsGlutaredoxinsBase SequenceAscorbic AcidCytosolPyrrolidonecarboxylic AcidMetabolism, Inborn ErrorsMagnetic Resonance SpectroscopyCell LineOxidantsThiobarbituric Acid Reactive SubstancesMutationCarbon RadioisotopesGene Expression Regulation, EnzymologicPeroxidesXenobioticsMass SpectrometryInsulinCarbon IsotopesAldehydesCarbonBrainLipid PeroxidesLactic AcidEnzyme InductionKidneyOxygenAdenosine TriphosphateDiamideThioredoxinsEthacrynic AcidAryl Hydrocarbon Hydroxylasestert-ButylhydroperoxideHomeostasisS-NitrosoglutathioneDietCytochrome P-450 CYP3ARecombinant ProteinsEscherichia coliNitrogenProtein BindingEnzyme Inhibitors
PeroxidaseMetabolicCysteineGlutamateMethionine metabolismEnzymeGamma-glutamylcyPathwaySynthetaseAntioxidantGlucose MetabolismInbornMiceSerineGlycineMetabolitesMitochondrialXenobioticsDeficiencyInflammationGlycolysisCytochromeDetoxificationGSSGOxidantAmino acidDisordersLiverEnhancesDepletionGSTsReductaseEstrogen metabolismDispositionApoptosisCompoundsProteinsGeneticSkeletalUrineAnalysesModifications
Peroxidase7
Metabolic5
  • Glutathione S-transferases (GSTs), previously known as ligandins, are a family of eukaryotic and prokaryotic phase II metabolic isozymes best known for their ability to catalyze the conjugation of the reduced form of glutathione (GSH) to xenobiotic substrates for the purpose of detoxification. (wikipedia.org)
  • Glutathione synthetase (GS) deficiency, first described in 1970, is a rare autosomal-recessive inborn error of glutathione metabolism characterized by severe metabolic acidosis , hemolytic anemia, and neurological problems. (medscape.com)
  • Altogether, these results show that glucose metabolism and glycogen utilization are dysregulated in astrocytes showing a chronic deficit in GSH, suggesting that alterations of a fundamental aspect of brain energy metabolism is caused by GSH deficit and may therefore be relevant to metabolic dysfunctions observed in schizophrenia. (edu.sa)
  • These training-related metabolic adaptations should increase glucose utilization and reduce intracellular glucose levels, thereby decreasing glucose metabolism via the polyol pathway. (hindawi.com)
  • Missense mutations in the human glutathione synthetase gene result in severe metabolic acidosis, 5-oxoprolinuria, hemolytic anemia and neurological dysfunction. (medscape.com)
Cysteine5
  • At intervals for 24 hr, samples of blood and other tissues were obtained, processed, and analyzed for reduced GSH, glutathione disulfide (GSSG), cysteine, and cystine using HPLC with dual electrochemical detection. (aspetjournals.org)
  • Two subgroups of cytosolic GSTs have been characterized based upon their interaction with glutathione: the Y-GST group, which uses a tyrosine residue to activate glutathione, and the S/C-GST, which instead uses serine or cysteine residues. (wikipedia.org)
  • Glutathione is produced from the amino acids cysteine, glycine, and glutamine via the consecutive actions of gamma-glutamylcysteine synthetase and glutathione synthetase. (medscape.com)
  • Atkuri KR, Mantovani JJ, Herzenberg LA, Herzenberg LA. N-Acetylcysteine--a safe antidote for cysteine/glutathione deficiency. (medscape.com)
  • Cysteine is then metabolized to sulfite, taurine, and glutathione. (msdmanuals.com)
Glutamate3
  • Proton MRS (1 H-MRS) enables in vivo measurement of small molecules implicated in multiple sclerosis, but its application to key metabolites glutamate, γ-aminobutyric acid (GABA), and glutathione has been sparse. (lu.se)
  • We employed, at 7 T, a previously validated 1 H-MRS protocol to measure glutamate, GABA, and glutathione, as well as glutamine, N-acetyl aspartate, choline, and myoinositol, in the frontal cortex of individuals with relapsing-remitting (N = 26) or progressive (N = 21) multiple sclerosis or healthy control adults (N = 25) in a cross-sectional analysis. (lu.se)
  • As a group, multiple sclerosis patients demonstrated significant negative correlations between disease duration and glutamate or GABA (ρ = -0.4, p = 0.02) but not glutamine or glutathione. (lu.se)
Methionine metabolism1
  • A number of defects in methionine metabolism lead to accumulation of homocysteine (and its dimer, homocystine) with adverse effects including thrombotic tendency, lens dislocation, and central nervous system and skeletal abnormalities. (msdmanuals.com)
Enzyme6
  • In mild glutathione synthetase deficiency, which is characterized by hemolytic anemia , enzyme deficiency primarily occurs in erythrocytes. (medscape.com)
  • We found upregulation of the key fructose transporter and metabolizing enzyme mRNAs, Slc2a2 , Khka , and Khkc , and higher ketohexokinase activity in the Hif-p4h-2 gt/gt small intestine relative to the WT, suggesting enhanced metabolism of fructose in the former. (springer.com)
  • ChaC2, an Enzyme for Slow Turnover of Cytosolic Glutathione. (nih.gov)
  • In cells , antioxidants such as glutathione , mycothiol or bacillithiol , and enzyme systems like superoxide dismutase , can prevent damage from oxidative stress . (wikipedia.org)
  • Furthermore, HIF-1 actively limits the mitochondrial consumption of pyruvate at two levels: (i) through the enzyme PDK (PDH-kinase), which in turn inhibits PDH activity preventing the conversion of pyruvate into acetyl CoA, and thereby limiting mitochondrial metabolism of pyruvate [ 6 ] and (ii) through the direct activation of LDH [ 7 , 8 ]. (hindawi.com)
  • acetaminophen will increase the level or effect of lonafarnib by affecting hepatic/intestinal enzyme CYP3A4 metabolism. (medscape.com)
Gamma-glutamylcy1
  • The activity of GSTs is dependent upon a steady supply of GSH from the synthetic enzymes gamma-glutamylcysteine synthetase and glutathione synthetase, as well as the action of specific transporters to remove conjugates of GSH from the cell. (wikipedia.org)
Pathway4
Synthetase12
  • Multiple mutations that cause glutathione synthetase deficiency have been described in the glutathione synthetase gene, GSS . (medscape.com)
  • Thirteen different missense mutations in GSS have been identified in individuals with severe glutathione synthetase deficiency. (medscape.com)
  • The frequency of glutathione synthetase deficiency is unknown. (medscape.com)
  • Glutathione synthetase deficiency is very rare. (medscape.com)
  • It is believed that some cases of glutathione synthetase deficiency may go undiagnosed or misdiagnosed, complicating a determination of its true frequency. (medscape.com)
  • Recently, authors have recommended that 3 forms of glutathione synthetase deficiency be identified: mild, moderate, and severe (see History). (medscape.com)
  • According to one review, the oldest reported survivor with the severe form of glutathione synthetase deficiency was aged 24 years and had experienced significant neurological deterioration over the previous few years. (medscape.com)
  • No race predilection is observed in glutathione synthetase deficiency. (medscape.com)
  • Most individuals with systemic glutathione synthetase deficiency are diagnosed in the newborn period. (medscape.com)
  • Available at https://rarediseases.org/rare-diseases/glutathione-synthetase-deficiency/ . (medscape.com)
  • Available at https://rarediseases.info.nih.gov/diseases/10047/glutathione-synthetase-deficiency . (medscape.com)
  • Diagnosis of glutathione synthetase deficiency in newborn screening. (medscape.com)
Antioxidant1
Glucose Metabolism1
  • This study aims at investigating glucose metabolism in cultured astrocytes from GCLM knockout mice, which show decreased GSH levels. (edu.sa)
Inborn2
  • Approach to the Patient With a Suspected Inherited Disorder of Metabolism Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. (msdmanuals.com)
  • Initial testing Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. (msdmanuals.com)
Mice4
  • Our previous finding of a glutathione (GSH) deficiency in aging or senescent mice suggested that a concomitant decrease in detoxification capacity also may occur. (aspetjournals.org)
  • Overall, humanized TK-NOG chimeric mice were considered to be a functional tool for the study of drug metabolism of diclofenac in humans. (aspetjournals.org)
  • D ) RNA-Seq-based GSEA for carbohydrate metabolism in the adrenal cortex of LPS versus PBS mice. (elifesciences.org)
  • E ) GSEA for proteins associated with carbohydrate metabolism in CD31 - CD45 - adrenocortical cells of LPS versus PBS mice. (elifesciences.org)
Serine1
  • Our analysis and metabolomic measurements showed that plasma levels of glycine, serine, and associated metabolites are negatively correlated with HS, suggesting that these GSH metabolism precursors might be limiting. (chalmers.se)
Glycine2
  • The tripeptide glutathione (GSH) or γ-glutamylcysteinyl-glycine is the most abundant low molecular weight thiol in most eukaryotic organisms and serves a number of important functions as sulfhydryl-buffer, cofactor for enzymes and for the detoxification of xenobiotics and drugs. (gla.ac.uk)
  • The region containing the greatest amount of variability between the assorted classes is that of helix α2, where one of three different amino acid residues interacts with the glycine residue of glutathione. (wikipedia.org)
Metabolites1
  • Diclofenac also produces glutathione-conjugated metabolites, some of which are human-specific. (aspetjournals.org)
Mitochondrial3
  • The glutathione binding site, or "G-site", is located in the thioredoxin-like domain of both cytosolic and mitochondrial GSTs. (wikipedia.org)
  • Mitochondrial metabolism is regulated oppositely in Alzheimer's disease and lung cancer, indicating that it may be involved in the inverse co-morbidity between these diseases. (nature.com)
  • In contrast to normal cells, most cancer cells predominantly produce energy by a high rate of glycolysis followed by lactate fermentation, even in the presence of oxygen, a less efficient metabolism compared to a low rate of glycolysis followed by mitochondrial oxidation of pyruvate [ 2 ]. (hindawi.com)
Xenobiotics2
Deficiency1
Inflammation1
  • Free radicals are generated in the CNS by ongoing oxygen metabolism and biological events associated with injury and inflammation. (utmb.edu)
Glycolysis2
  • These genes increase oxygen availability by inducing erythropoiesis and angiogenesis, and also reduce its demand via regulation of energy metabolism by reducing oxidative phosphorylation and inducing non-oxygen-demanding glycolysis [ 5 , 6 , 7 ]. (springer.com)
  • For example, endurance exercise training increases the synthesis and activity of hexokinase II [ 6 , 7 ], which increases the capacity of the skeletal muscle cell to phosphorylate glucose with subsequent metabolism through glycolysis. (hindawi.com)
Cytochrome2
  • Current in vitro studies were undertaken to probe the cytochrome P450 (P450)-mediated bioactivation of flutamide and identify the possible reactive species using reduced glutathione (GSH) as a trapping agent. (unboundmedicine.com)
  • DCEG investigators have evaluated the glutathione-S-transferase and cytochrome P-450 families of genes, both of which encode enzymes involved in the metabolism of solvents and other substrates. (cdc.gov)
Detoxification2
GSSG1
  • however, a tentative bioactivation mechanism involving a P450-catalyzed abstraction of hydrogen atom from the amide nitrogen of flutamide and the subsequent trapping of the nitrogen-centered radical by GSH or oxidized glutathione (GSSG) was proposed. (unboundmedicine.com)
Oxidant1
  • Pan, Z & Perez-Polo, R 1993, ' Role of nerve growth factor in oxidant homeostasis: Glutathione metabolism ', Journal of Neurochemistry , vol. 61, no. 5, pp. 1713-1721. (utmb.edu)
Amino acid2
Disorders1
  • Neurodegenerative and psychiatric disorders including Alzheimer's, Parkinson's or Huntington's diseases and schizophrenia have been associated with a deficit in glutathione (GSH). (edu.sa)
Liver4
Enhances2
  • It is thought that this gene is upregulated in response to endoplasmic reticulum stress and that the glutathione depletion enhances apoptosis. (nih.gov)
  • The component increases metabolism and enhances the liver's ability to absorb lipid molecules. (mid-day.com)
Depletion1
  • This hypothesis was tested using acrylonitrile (ACN), a widely used industrial chemical whose metabolism is associated with glutathione (GSH) depletion and cyanide (CN) generation. (cdc.gov)
GSTs1
  • Glutathione S-transferases (GSTs), a superfamily of multifunctional enzymes, play an important role in the onset and progression of renal cell carcinoma (RCC). (cancerindex.org)
Reductase1
  • The same procedure was carried out with femoral muscle tissue.It was found that allotransplantation of the embryo muscle tissue leads to an increasing of restored glutathione at day 7 of experiment in femoral and abdominal muscle of adult rat, leads the increasing of glutathione glutathione reductase activity in all researched tissues on day 7 of the experiment, except the femoral muscle of the adult rat. (eujournal.org)
Estrogen metabolism1
Disposition3
Apoptosis1
Compounds2
  • Melatonin may exert some effects on the activity of enzymes engaged in the metabolism of thiol compounds in the cell. (nih.gov)
  • We treated mouse cholangiocytes in three-dimensional (3D) spheroid culture and neonatal extrahepatic duct explants with biliatresone and compounds that regulate glutathione (GSH). (nih.gov)
Proteins1
  • 2. "Self'" molecules (peptides/proteins) in the lower airways, susceptible to chemical reactivity with HDI, have been hypothesized to play a role in asthma pathogenesis and/ or chemical metabolism, but remain poorly characterized. (cdc.gov)
Genetic2
  • In contrast, cancer cells shift their metabolism toward lactate production even in the presence of oxygen [ 4 ], partly through genetic modifications that stabilize the transcription factor Hypoxia Inducible Factor (HIF) involved in the adaptation of the cells to hypoxia, under nonhypoxic conditions as well as generating an adaptive response to the hypoxic microenvironment (Figure 1 ). (hindawi.com)
  • Increased N7-methyldeoxyguanosine DNA adducts after occupational exposure to pesticides and influence of genetic polymorphisms of paraoxonase-1 and glutathione S-transferase M1 and T1. (cdc.gov)
Skeletal1
  • Therefore, sorbitol metabolism may contribute to alterations by which skeletal muscle strength is decreased in patients with long-term DM. (hindawi.com)
Urine1
  • The majority of TCE absorbed into the body is eliminated after metabolism through the urine and feces. (cdc.gov)
Analyses1
Modifications1
  • Modifications in the metabolism of glucose induced by endurance exercise training have the potential to affect the concentrations of sorbitol in diabetic individuals too. (hindawi.com)