OrganismsChemicals and DrugsPhenomena and Processes
Glycine DehydrogenaseGlycine Dehydrogenase (Decarboxylating)GlycineMalate DehydrogenaseDecarboxylationSelenomonasIsocitrate DehydrogenasePhosphogluconate Dehydrogenase
Cleavage system P proteinEnzymeGLDCEncephalopathyDecarboxylase ComplexGlutamate dehydrogenasePyruvateNonketotic hyperglycinemiaMitochondrialAminoEnzymesAlanineDegradationMolecularEnsemblDisulfideCysteineLiverTissuesSuggestsYieldNormalCleavageDecarboxylase complexPyruvate dehydrogenaseSynthaseGcvPPyridoxal phosphateGeneTissuesComponentsBodyBrainCurrent
Cleavage system P protein1
Enzyme10
  • Glycine decarboxylase (EC 1.4.4.2) is an enzyme that catalyzes the following chemical reaction: glycine + H-protein-lipoyllysine ⇌ {\displaystyle \rightleftharpoons } H-protein-S-aminomethyldihydrolipoyllysine + CO2 Thus, the two substrates of this enzyme are glycine and H-protein-lipoyllysine, whereas its two products are H-protein-S-aminomethyldihydrolipoyllysine and CO2. (wikipedia.org)
  • This enzyme participates in glycine, serine and threonine metabolism. (wikipedia.org)
  • Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). (wikipedia.org)
  • Glycine decarboxylase, or P-protein, is a pyridoxal 5'-phosphate (PLP)-dependent enzyme in one-carbon metabolism of all organisms, in the glycine and serine catabolism of vertebrates, and in the photorespiratory pathway of oxygenic phototrophs. (nih.gov)
  • PLP and the substrate glycine bind in the active site of this reduced enzyme and appear to cause further conformational changes involving a flexible surface loop. (nih.gov)
  • The GLDC gene provides instructions for making an enzyme called glycine dehydrogenase. (medlineplus.gov)
  • When an altered version of this enzyme is incorporated into the glycine cleavage system, it impairs the system's ability to break down glycine. (medlineplus.gov)
  • 3. Pahlich, E. and Joy, K.W. Glutamate dehydrogenase from pea roots: purification and properties of the enzyme. (qmul.ac.uk)
  • What enzyme decarboxylates the α-keto acids of branched-chain amino acids? (flashcardmachine.com)
  • OMP is then decarboxylated by the enzyme OMP decarboxylase to yield uridine monophosphate (UMP). (biologyonline.com)
GLDC4
  • Glycine encephalopathy is due to defects in GLDC or AMT of the glycine cleavage system. (wikipedia.org)
  • Other mutations insert or delete genetic material in the GLDC gene, or disrupt how genetic information from the gene is spliced together to make a blueprint for producing glycine dehydrogenase. (medlineplus.gov)
  • Some GLDC gene mutations lead to the production of a nonfunctional version of glycine dehydrogenase, while other mutations reduce but do not eliminate the enzyme's activity. (medlineplus.gov)
  • Conter C, Rolland MO, Cheillan D, Bonnet V, Maire I, Froissart R. Genetic heterogeneity of the GLDC gene in 28 unrelated patients with glycine encephalopathy. (medlineplus.gov)
Encephalopathy1
  • Applegarth DA, Toone JR. Glycine encephalopathy (nonketotic hyperglycinemia): comments and speculations. (medlineplus.gov)
Decarboxylase Complex1
Glutamate dehydrogenase3
  • 1. Frieden, C. L -Glutamate dehydrogenase. (qmul.ac.uk)
  • Ammonia incorporation in animals occurs through the actions of glutamate dehydrogenase and glutamine synthase. (medmuv.com)
  • A pair of principal enzymes, glutamate dehydrogenase and glutamine synthatase, are found in all organisms and effect the conversion of ammonia into the amino acids glutamate and glutamine, respectively. (medmuv.com)
Pyruvate2
  • As a therapeutic, its principal mechanism of action is to inhibit pyruvate dehydrogenase kinase (PDK). (srcinhibitors.com)
  • In turn, PDK inhibits the key mitochondrial energy homeostat, pyruvate dehydrogenase complex (PDC), by reversible phosphorylation. (srcinhibitors.com)
Nonketotic hyperglycinemia1
Mitochondrial2
  • The mitochondrial glycine cleavage system. (wikipedia.org)
  • Lastly , the dihydroorotate is oxidized by dihydroorotate dehydrogenase (an integral membrane protein in the inner mitochondrial membrane ) to convert into orotate . (biologyonline.com)
Amino5
  • The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor. (wikipedia.org)
  • Glycine is an amino acid, which is a building block of proteins. (medlineplus.gov)
  • Many of these genetic changes alter single amino acids in glycine dehydrogenase. (medlineplus.gov)
  • IMP, in turn, is produced from a pre-existing ribose phosphate that forms mainly from the amino acids glycine , glutamine , and aspartic acid . (biologyonline.com)
  • Subsequently, L -DOPA is decarboxylated to DA by aromatic amino acid decarboxylase [ 21 ]. (biomedcentral.com)
Enzymes2
  • This protein is one of four enzymes that work together in a group called the glycine cleavage system. (medlineplus.gov)
  • Enzymes of neuronal energetic pathways, such as aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactate dehydrogenase (LDH), were also evaluated. (hindawi.com)
Alanine1
  • 1. O'Connor, R.J. and Halvorson, H. The substrate specificity of L -alanine dehydrogenase. (qmul.ac.uk)
Degradation1
Molecular1
  • The observation of the disulfide bond that acts to stabilize the closed form suggests a molecular mechanism for the redox-dependent activation of glycine decarboxylase observed earlier. (nih.gov)
Ensembl2
  • Acyl-CoA dehydrogenase [Ensembl]. (ntu.edu.sg)
  • Possible acyl-CoA dehydrogenase FadE19 (MMGC) [Ensembl]. (ntu.edu.sg)
Disulfide1
  • The presence of the disulfide bridge isolates the active site from solvent and hinders the binding of PLP and glycine in the active site. (nih.gov)
Cysteine1
  • Cysteine sulfinic acid, in turn, is decarboxylated by sulfinoalanine decarboxylase to form hypotaurine . (cloudfront.net)
Liver1
Tissues1
Suggests1
  • As its name suggests, the glycine cleavage system breaks down a molecule called glycine by cutting (cleaving) it into smaller pieces. (medlineplus.gov)
Yield1
  • Hypotaurine is enzymatically oxidized to yield taurine by hypotaurine dehydrogenase . (cloudfront.net)
Normal1
  • The breakdown of excess glycine when it is no longer needed is necessary for the normal development and function of nerve cells in the brain. (medlineplus.gov)
Cleavage9
  • Glycine decarboxylase is the P-protein of the glycine cleavage system in eukaryotes. (wikipedia.org)
  • The glycine cleavage system catalyzes the degradation of glycine. (wikipedia.org)
  • The glycine cleavage system. (wikipedia.org)
  • 1981). "Defective glycine cleavage system in nonketotic hyperglycinemia. (wikipedia.org)
  • This protein is one of four enzymes that work together in a group called the glycine cleavage system. (medlineplus.gov)
  • As its name suggests, the glycine cleavage system breaks down a molecule called glycine by cutting (cleaving) it into smaller pieces. (medlineplus.gov)
  • The breakdown of glycine by the glycine cleavage system produces a molecule called a methyl group. (medlineplus.gov)
  • When an altered version of this enzyme is incorporated into the glycine cleavage system, it impairs the system's ability to break down glycine. (medlineplus.gov)
  • Nonketotic hyperglycinemia (NKH) is the inborn error of glycine metabolism defined by deficient activity of the glycine cleavage enzyme system (GCS), which results in accumulation of large quantities of glycine in all body tissues including the brain. (nih.gov)
Decarboxylase complex2
  • A PYRIDOXAL PHOSPHATE dependent enzyme that catalyzes the decarboxylation of GLYCINE with the transfer of an aminomethyl group to the LIPOIC ACID moiety of the GLYCINE DECARBOXYLASE COMPLEX H-PROTEIN . (nih.gov)
  • It is one of four subunits of the glycine decarboxylase complex. (nih.gov)
Pyruvate dehydrogenase1
  • The crystal structure of dihydrolipoamide dehydrogenase and dihydrolipoamide dehydrogenase-binding protein (didomain) subcomplex of human pyruvate dehydrogenase complex. (yeastrc.org)
Synthase1
  • Ammonia incorporation in animals occurs through the actions of glutamate dehydrogenase and glutamine synthase. (tdmuv.com)
GcvP1
Pyridoxal phosphate1
  • The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor. (wikipedia.org)
Gene1
  • The protein encoded by this gene is the P protein, which binds to glycine and enables the methylamine group from glycine to be transferred to the T protein. (nih.gov)
Tissues1
Components1
  • GSH that the substrate of GST and its components including glutamic acid, cysteine and glycine accumulated in transgenic rice. (bvsalud.org)
Body1
  • This condition is characterized by abnormally high levels of glycine in the body (hyperglycinemia). (medlineplus.gov)
Brain1
  • The breakdown of excess glycine when it is no longer needed is necessary for the normal development and function of nerve cells in the brain. (medlineplus.gov)
Current1
  • Current treatment is reduction of plasma concentration of glycine by administration of sodium benzoate and blockade of overstimulated NMDA receptors. (nih.gov)