carbamyl phosphate

Summary

Summary: Phosphate salt of carbamic acid.

Top Publications

  1. ncbi Catalytic domains of carbamyl phosphate synthetase. Glutamine-hydrolyzing site of Escherichia coli carbamyl phosphate synthetase
    S D Rubino
    J Biol Chem 261:11320-7. 1986
  2. ncbi Crystal structure of human ornithine transcarbamylase complexed with carbamoyl phosphate and L-norvaline at 1.9 A resolution
    D Shi
    Department of Biochemistry, University of Minnesota, St Paul, Minnesota, USA
    Proteins 39:271-7. 2000
  3. ncbi Metabolic channeling of carbamoyl phosphate, a thermolabile intermediate: evidence for physical interaction between carbamate kinase-like carbamoyl-phosphate synthetase and ornithine carbamoyltransferase from the hyperthermophile Pyrococcus furiosus
    Jan Massant
    Department of Microbiology, Vrije Universiteit Brussel, Flanders Interuniversity Institute for Biotechnology, Universite Libre de Bruxelles, Belgium
    J Biol Chem 277:18517-22. 2002
  4. pmc Structural model of the R state of Escherichia coli aspartate transcarbamoylase with substrates bound
    Jie Wang
    Department of Chemistry, Boston College, Merkert Chemistry Center, Chestnut Hill, MA 02467, USA
    J Mol Biol 371:1261-73. 2007
  5. ncbi The crystal structures of ornithine carbamoyltransferase from Mycobacterium tuberculosis and its ternary complex with carbamoyl phosphate and L-norvaline reveal the enzyme's catalytic mechanism
    Ramasamy Sankaranarayanan
    Group in Protein Structure and Function, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
    J Mol Biol 375:1052-63. 2008
  6. doi Insights into the mechanism and regulation of pyruvate carboxylase by characterisation of a biotin-deficient mutant of the Bacillus thermodenitrificans enzyme
    Abdussalam Adina-Zada
    School of Biomedical, Biomolecular and Chemical Sciences, The University of Western Australia, 35 Stirling Highway, Crawley, WA 6009, Australia
    Int J Biochem Cell Biol 40:1743-52. 2008
  7. ncbi Molecular physiology of phosphoryl group transfer from carbamoyl phosphate by a hyperthermophilic enzyme at low temperature
    Santiago Ramon-Maiques
    Instituto de Biomedicina de Valencia CSIC, Jaume Roig 11, 46010 Valencia, Spain
    Biochemistry 41:3916-24. 2002
  8. ncbi A functional analysis of the allosteric nucleotide monophosphate binding site of carbamoyl phosphate synthetase
    Olivier A Pierrat
    Department of Chemistry, Texas A and M University, College Station, Texas, 77842 3012
    Arch Biochem Biophys 400:34-42. 2002
  9. pmc A 70-amino acid zinc-binding polypeptide fragment from the regulatory chain of aspartate transcarbamoylase causes marked changes in the kinetic mechanism of the catalytic trimer
    B B Zhou
    Department of Molecular and Cell Biology, University of California, Berkeley 94720
    Protein Sci 3:967-74. 1994
  10. ncbi Kinetics of the quaternary structure change of aspartate transcarbamylase triggered by succinate, a competitive inhibitor
    H Tsuruta
    Stanford Synchrotron Radiation Laboratory, SLAC, California 94309 0210
    Biochemistry 33:10007-12. 1994

Research Grants

Detail Information

Publications184 found, 100 shown here

  1. ncbi Catalytic domains of carbamyl phosphate synthetase. Glutamine-hydrolyzing site of Escherichia coli carbamyl phosphate synthetase
    S D Rubino
    J Biol Chem 261:11320-7. 1986
    We present evidence that cysteine 269 of the small subunit of Escherichia coli carbamyl phosphate synthetase is essential for the hydrolysis of glutamine...
  2. ncbi Crystal structure of human ornithine transcarbamylase complexed with carbamoyl phosphate and L-norvaline at 1.9 A resolution
    D Shi
    Department of Biochemistry, University of Minnesota, St Paul, Minnesota, USA
    Proteins 39:271-7. 2000
    ..The structures of the active sites in the complexes of aspartate transcarbamylase (ATCase) with various substrates or inhibitors are similar to this OTCase structure, consistent with their common evolutionary origin...
  3. ncbi Metabolic channeling of carbamoyl phosphate, a thermolabile intermediate: evidence for physical interaction between carbamate kinase-like carbamoyl-phosphate synthetase and ornithine carbamoyltransferase from the hyperthermophile Pyrococcus furiosus
    Jan Massant
    Department of Microbiology, Vrije Universiteit Brussel, Flanders Interuniversity Institute for Biotechnology, Universite Libre de Bruxelles, Belgium
    J Biol Chem 277:18517-22. 2002
    ..Therefore, by physically interacting with each other, CKase and OTCase prevent the thermodenaturation of carbamoyl phosphate in the aqueous cytoplasmic environment...
  4. pmc Structural model of the R state of Escherichia coli aspartate transcarbamoylase with substrates bound
    Jie Wang
    Department of Chemistry, Boston College, Merkert Chemistry Center, Chestnut Hill, MA 02467, USA
    J Mol Biol 371:1261-73. 2007
    ..By combining these data with previously determined structures we can now visualize the allosteric transition through detailed atomic motions that underlie the molecular mechanism...
  5. ncbi The crystal structures of ornithine carbamoyltransferase from Mycobacterium tuberculosis and its ternary complex with carbamoyl phosphate and L-norvaline reveal the enzyme's catalytic mechanism
    Ramasamy Sankaranarayanan
    Group in Protein Structure and Function, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
    J Mol Biol 375:1052-63. 2008
    ..We have interpreted this deprotonation as part of the collapse of the transition state of the reaction...
  6. doi Insights into the mechanism and regulation of pyruvate carboxylase by characterisation of a biotin-deficient mutant of the Bacillus thermodenitrificans enzyme
    Abdussalam Adina-Zada
    School of Biomedical, Biomolecular and Chemical Sciences, The University of Western Australia, 35 Stirling Highway, Crawley, WA 6009, Australia
    Int J Biochem Cell Biol 40:1743-52. 2008
    ..Attempts to trap the putative carboxyphosphate intermediate in K1112A using diazomethane were unsuccessful...
  7. ncbi Molecular physiology of phosphoryl group transfer from carbamoyl phosphate by a hyperthermophilic enzyme at low temperature
    Santiago Ramon-Maiques
    Instituto de Biomedicina de Valencia CSIC, Jaume Roig 11, 46010 Valencia, Spain
    Biochemistry 41:3916-24. 2002
    ..Probably the catalysis of the phosphoryl group transfer is less efficient at low temperature, as suggested by structural data showing that Lys131 is improperly positioned to assist the transfer...
  8. ncbi A functional analysis of the allosteric nucleotide monophosphate binding site of carbamoyl phosphate synthetase
    Olivier A Pierrat
    Department of Chemistry, Texas A and M University, College Station, Texas, 77842 3012
    Arch Biochem Biophys 400:34-42. 2002
    ..With the exception of V994A, these mutations had a modest influence on the binding and subsequent allosteric effects by UMP and IMP...
  9. pmc A 70-amino acid zinc-binding polypeptide fragment from the regulatory chain of aspartate transcarbamoylase causes marked changes in the kinetic mechanism of the catalytic trimer
    B B Zhou
    Department of Molecular and Cell Biology, University of California, Berkeley 94720
    Protein Sci 3:967-74. 1994
    ..The rate of dissociation of carbamoyl phosphate from the Zn domain-C trimer complex was about 10(-2) that from C trimer.(ABSTRACT TRUNCATED AT 250 WORDS)..
  10. ncbi Kinetics of the quaternary structure change of aspartate transcarbamylase triggered by succinate, a competitive inhibitor
    H Tsuruta
    Stanford Synchrotron Radiation Laboratory, SLAC, California 94309 0210
    Biochemistry 33:10007-12. 1994
    ..This is consistent with a concerted structural transition of ATCase.(ABSTRACT TRUNCATED AT 250 WORDS)..
  11. ncbi Apparent cooperativity for carbamoylphosphate in Escherichia coli aspartate transcarbamoylase only reflects cooperativity for aspartate
    P England
    Laboratoire de Biochimie des Signaux Régulateurs Cellulaires et Moléculaires, URA CNRS 1682, Universite Pierre et Marie Curie, Paris, France
    Eur J Biochem 222:775-80. 1994
    ....
  12. pmc Ligation alters the pathway of urea-induced denaturation of the catalytic trimer of Escherichia coli aspartate transcarbamylase
    S Bromberg
    Department of Biochemistry, University of Minnesota, St Paul 55108
    Protein Sci 3:1236-44. 1994
    ..c3) of aspartate transcarbamylase from Escherichia coli at low temperature in the absence and presence of carbamyl phosphate (CP; a substrate), N-(phosphonacetyl)-L-Asp (PALA; a bisubstrate analog), and 2 anionic inhibitors, Cl- and ..
  13. ncbi Maturation of [NiFe]-hydrogenases in Escherichia coli: the HypC cycle
    Melanie Blokesch
    Microbiology, Department I Biology, University of Munich, Maria Ward Strasse 1a, D 80638 Munich, Germany
    J Mol Biol 324:287-96. 2002
    ..On the basis of the results, a cycle of HypC activity is proposed whose function is to transfer the iron atom that has been liganded at the HypC-HypD complex to the precursor of the large hydrogenase subunit...
  14. ncbi Carbamoylphosphate serves as the source of CN(-), but not of the intrinsic CO in the active site of the regulatory [NiFe]-hydrogenase from Ralstonia eutropha
    Oliver Lenz
    Humboldt Universitat zu Berlin, Institut für Biologie Mikrobiologie, Chausseestrasse 117, Berlin, Germany
    FEBS Lett 581:3322-6. 2007
    ..Incorporation of label from (13)CO exclusively into the carbonyl ligand indicates that free CO is a possible precursor in carbonyl ligand biosynthesis...
  15. ncbi Deconstruction of the catalytic array within the amidotransferase subunit of carbamoyl phosphate synthetase
    X Huang
    Department of Chemistry, Texas A and M University, College Station, Texas, 77843, USA
    Biochemistry 38:15909-14. 1999
    ..It is concluded that contrary to the original proposal for the catalytic triad, Glu-355 is not an essential residue for catalysis. The results are consistent with Ser-47 and Gln-273 playing significant roles in the binding of glutamine...
  16. pmc Acetylornithine transcarbamylase: a novel enzyme in arginine biosynthesis
    Hiroki Morizono
    Children s Research Institute, Children s National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010, USA
    J Bacteriol 188:2974-82. 2006
    ..coli and show that it catalyzes the formation of N-acetyl-L-citrulline from N-acetyl-L-ornithine and carbamyl phosphate. We now designate this enzyme as an acetylornithine transcarbamylase...
  17. ncbi The 1.5 A resolution crystal structure of the carbamate kinase-like carbamoyl phosphate synthetase from the hyperthermophilic Archaeon pyrococcus furiosus, bound to ADP, confirms that this thermostable enzyme is a carbamate kinase, and provides insight in
    S Ramon-Maiques
    Consejo Superior de Investigaciones Cientificas IBV CSIC, Instituto de Biomedicina de Valencia, C Jaime Roig 11, Valencia, 46010, Spain
    J Mol Biol 299:463-76. 2000
    ..The structure provides the first information on substrate binding and catalysis in CKs, and suggests that the slow rate at 37 degrees C is possibly a consequence of slow product dissociation...
  18. ncbi Perforation of the tunnel wall in carbamoyl phosphate synthetase derails the passage of ammonia between sequential active sites
    Jungwook Kim
    Department of Chemistry, Texas A and M University, P O Box 30012, College Station, Texas 77842 3012, USA
    Biochemistry 43:5334-40. 2004
    ..Overall, the catalytic constants of the mutant were consistent with the model where the channeling of ammonia has been disrupted due to the leakage from the ammonia tunnel to the protein exterior...
  19. ncbi Half of Saccharomyces cerevisiae carbamoyl phosphate synthetase produces and channels carbamoyl phosphate to the fused aspartate transcarbamoylase domain
    V Serre
    Laboratoire de Biochimie des Signaux Régulateurs Cellulaires et Moléculaires, UMR 7631 CNRS Université Pierre et Marie Curie, 96 Bd Raspail 75006 Paris, France
    J Biol Chem 274:23794-801. 1999
    ....
  20. ncbi Molecular structure of dihydroorotase: a paradigm for catalysis through the use of a binuclear metal center
    J B Thoden
    Department of Biochemistry, University of Wisconsin, Madison, Wisconsin 53706, USA
    Biochemistry 40:6989-97. 2001
    ..The ensuing tetrahedral intermediate collapses with C-O bond cleavage and expulsion of the hydroxide which then bridges the binuclear metal center...
  21. pmc Carbamate kinase: New structural machinery for making carbamoyl phosphate, the common precursor of pyrimidines and arginine
    A Marina
    Instituto de Biomedicina de Valencia CSIC, Spain
    Protein Sci 8:934-40. 1999
    ..The orientation of the crevices in the dimer precludes their physical cooperation in the catalytic process. Such cooperation is not needed in the CK reaction but is a requirement of the mechanism of CPSs...
  22. ncbi The structure of carbamoyl phosphate synthetase determined to 2.1 A resolution
    J B Thoden
    Institute for Enzyme Research, The Graduate School, and Department of Biochemistry, College of Agricultural and Life Sciences, University of Wisconsin Madison, 1710 University Avenue, Madison, Wisconsin 53705, USA
    Acta Crystallogr D Biol Crystallogr 55:8-24. 1999
    ..The carboxyphosphate and carbamoyl phosphate synthetic components, while topologically equivalent, are structurally different, as would be expected in light of their separate biochemical functions...
  23. ncbi cDNA cloning of two isoforms of ornithine carbamoyltransferase from Canavalia lineata leaves and the effect of site-directed mutagenesis of the carbamoyl phosphate binding site
    Y Lee
    School of Biological Sciences, Seoul National University, Korea
    Plant Mol Biol 46:651-60. 2001
    ..It is assumed that the substrate specificity of ClOCT1 or ClOCT2 proteins partially depends on the amino acid sequence of the CP-binding site...
  24. ncbi Divergent polyamine metabolism in the Apicomplexa
    Tuesday Cook
    Haskins Laboratories, Pace University, New York, NY 10038, USA
    Microbiology 153:1123-30. 2007
    ..The lack of polyamine biosynthesis by T. gondii is contrasted with polyamine metabolism by other apicomplexans...
  25. ncbi Direct observation of an altered quaternary-structure transition in a mutant aspartate transcarbamoylase
    H Tsuruta
    Stanford Synchrotron Radiation Laboratory, California, USA
    Proteins 31:383-90. 1998
    ..They also illustrate the crucial role played by domain motions and quaternary-structure changes for both the homotropic and heterotropic aspects of allostery...
  26. ncbi Tertiary and quaternary conformational changes in aspartate transcarbamylase: a normal mode study
    A Thomas
    Laboratoire de Dynamique Moleculaire, Institut de Biologie Structurale Jean Pierre Ebel, Grenoble, France
    Proteins 34:96-112. 1999
    ..The particular dynamic behavior of these residues is examined in the light of biochemical results to obtain insights into their role in the transmission of the allosteric signal...
  27. pmc Isolation and partial characterization of an argR mutant of Salmonella typhimurium
    R A Kelln
    J Bacteriol 128:528-35. 1976
    ..Ingraham, Abstr. Annu. Meet. Am. Soc. Microbiol. 1975, K169, p. 175) have shown constitutive synthesis of carbamyl phosphate synthetase in the argR mutant, the regulation of the synthesis of the last five enzymes of the pyrimidine ..
  28. ncbi Importance of ornithine transcarbamylase (OTC) deficiency in small intestine for urinary orotic acid excretion: analysis of OTC-deficient spf-ash mice with OTC transgene
    T Saheki
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Japan
    Biochim Biophys Acta 1270:87-93. 1995
    ..These results suggest that the level of OTC activity in the small intestine is important for production of orotic acid...
  29. pmc Extent of genetic lesions of the arginine and pyrimidine biosynthetic pathways in Lactobacillus plantarum, L. paraplantarum, L. pentosus, and L. casei: prevalence of CO(2)-dependent auxotrophs and characterization of deficient arg genes in L. plantarum
    Françoise Bringel
    Laboratoire de Dynamique, Expression et Evolution des génomes de micro organismes, FRE 2326 Université Louis Pasteur CNRS, 28 rue Goethe, 67083 Strasbourg, France
    Appl Environ Microbiol 69:2674-83. 2003
    ..These HCR auxotrophs may have had their CP cellular pool-related genes altered or deregulated...
  30. ncbi Taming of a poison: biosynthesis of the NiFe-hydrogenase cyanide ligands
    Stefanie Reissmann
    Department Biologie I, Mikrobiologie, University of Munich, Maria Ward Strasse 1a, D 80638 Munich, Germany
    Science 299:1067-70. 2003
    ..This finding underscores a striking parallel between biochemistry and organometallic chemistry in the formation of an iron-cyano complex...
  31. ncbi Site-directed mutagenesis of Arg60 and Cys271 in ornithine transcarbamylase from rat liver
    S McDowall
    Department of Biochemistry, La Trobe University, Bundoora, Victoria, Australia
    Protein Eng 4:73-7. 1990
    ..The small loss in binding energy and relatively high residual catalytic activity of the mutant strongly suggests that a number of other residues are involved in the binding of ornithine.(ABSTRACT TRUNCATED AT 250 WORDS)..
  32. pmc New experimental approaches for investigating interactions between Pyrococcus furiosus carbamate kinase and carbamoyltransferases, enzymes involved in the channeling of thermolabile carbamoyl phosphate
    Jan Massant
    Laboratorium voor Erfelijkheidsleer en Microbiologie, Vrije Universiteit Brussel, Pleinlaan 2, B 1050 Brussel, Belgium
    Archaea 1:365-73. 2005
    ....
  33. ncbi Metabolic channelling of carbamoyl phosphate in the hyperthermophilic archaeon Pyrococcus furiosus: dynamic enzyme-enzyme interactions involved in the formation of the channelling complex
    J Massant
    Laboratorium voor Erfelijkheidsleer en Microbiologie, Vrije Universiteit Brussel, Pleinlaan 2, B 1050 Brussels, Belgium
    Biochem Soc Trans 32:306-9. 2004
    ....
  34. pmc In Pseudomonas syringae pv. phaseolicola, expression of the argK gene, encoding the phaseolotoxin-resistant ornithine carbamoyltransferase, is regulated indirectly by temperature and directly by a precursor resembling carbamoylphosphate
    Karina López-López
    Departamento de Ingeniería Genética, Centro de Investigación y de Estudios Avanzados CINVESTAV del Instituto Politecnico Nacional IPN, Unidad Irapuato, Irapuato, Guanajuato, C P 36500, Mexico
    J Bacteriol 186:146-53. 2004
    ..These results indicate that argK expression is independent of temperature and is regulated directly by a compound resembling the inorganic moiety of phaseolotoxin...
  35. ncbi Kinetic characterization of yeast pyruvate carboxylase isozyme Pyc1 and the Pyc1 mutant, C249A
    Joy P Branson
    Biochemistry and Molecular Biology, School of Biomedical and Chemical Sciences, The University of Western Australia, 35 Stirling Highway, Crawley, WA 6009, Australia
    Biochemistry 43:1075-81. 2004
    ....
  36. pmc Three-dimensional structure of carbamoyl phosphate and succinate bound to aspartate carbamoyltransferase
    J E Gouaux
    Gibbs Chemical Laboratory, Harvard University, Cambridge, MA 02138
    Proc Natl Acad Sci U S A 85:4205-8. 1988
    ..In comparing the structure of the active sites in the PALA-enzyme complex to the active sites in the carbamoyl phosphate-succinate-enzyme complex, we find that they are similar...
  37. ncbi Regulatory changes in the control of carbamoyl phosphate synthetase induced by truncation and mutagenesis of the allosteric binding domain
    R M Czerwinski
    Department of Chemistry, Texas A and M University, College Station 77843, USA
    Biochemistry 34:13920-7. 1995
    ..Six conserved amino acids within the allosteric domain were mutated.(ABSTRACT TRUNCATED AT 250 WORDS)..
  38. ncbi HypF, a carbamoyl phosphate-converting enzyme involved in [NiFe] hydrogenase maturation
    Athanasios Paschos
    Mikrobiologie, Department I der Fakultät für Biologie, Ludwig Maximilians Universitat Munchen, Maria Ward Strasse 1a, D 80638 Munich, Germany
    J Biol Chem 277:49945-51. 2002
    ..The results are discussed in terms of the involvement of HypF in the conversion of carbamoyl phosphate to the CN ligand...
  39. ncbi T-state active site of aspartate transcarbamylase: crystal structure of the carbamyl phosphate and L-alanosine ligated enzyme
    Jingwei Huang
    Department of Chemistry and Chemical Biology, Harvard University, Cambridge, Massachusetts 02138, USA
    Biochemistry 45:346-52. 2006
    ..2 A) when ATCase is bound to carbamyl phosphate (CP) and to L-alanosine (an analogue of aspartate)...
  40. ncbi Products in the T-state of aspartate transcarbamylase: crystal structure of the phosphate and N-carbamyl-L-aspartate ligated enzyme
    Jingwei Huang
    Department of Chemistry and Chemical Biology, Harvard University, Cambridge, Massachusetts 02138, USA
    Biochemistry 43:6422-6. 2004
    ..the transition state like those found in our earlier studies of other ligands (N-phosphonacetyl-L-aspartate, carbamyl phosphate plus malonate, phosphonoacetamide plus malonate, or citrate plus phosphate)...
  41. pmc Weakening of the interface between adjacent catalytic chains promotes domain closure in Escherichia coli aspartate transcarbamoylase
    D P Baker
    Merkert Chemistry Center, Department of Chemistry, Boston College, Chestnut Hill, Massachusetts 02167 3860, USA
    Protein Sci 4:258-67. 1995
    ..Solution X-ray scattering was also used to investigate the structural consequences of nucleotide binding to the Glu-50/Asp-100-->Ala enzyme...
  42. ncbi Aspartate transcarbamylase from the hyperthermophilic archaeon Pyrococcus abyssi. Insights into cooperative and allosteric mechanisms
    Sigrid Van Boxstael
    Erfelijkheidsleer en Microbiologie, Vrije Universiteit Brussel, Belgium
    FEBS J 272:2670-83. 2005
    ..In contrast to the same enzyme in P. abyssi cellular extracts, the pure enzyme is inhibited by the carbamyl phosphate analogue phosphonacetate; this difference supports the idea that in native cells ATCase interacts with ..
  43. ncbi Structural defects within the carbamate tunnel of carbamoyl phosphate synthetase
    Jungwook Kim
    Department of Chemistry, Texas A and M University, P O Box 30012, College Station, Texas 77843 3012, USA
    Biochemistry 41:12575-81. 2002
    ..The partial reactions of CPS have not been significantly impaired by these mutations, and thus, the catalytic machinery at the individual active sites has not been functionally perturbed...
  44. pmc The carbamate kinase-like carbamoyl phosphate synthetase of the hyperthermophilic archaeon Pyrococcus furiosus, a missing link in the evolution of carbamoyl phosphate biosynthesis
    V Durbecq
    Laboratoire de Microbiologie, Universite Libre de Bruxelles, Brussels, Belgium
    Proc Natl Acad Sci U S A 94:12803-8. 1997
    ..The gene coding for this CK-like CPS was called cpkA...
  45. ncbi Thermodynamics of the reactions of carbamoyl phosphate
    Robert A Alberty
    Department of Chemistry, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Arch Biochem Biophys 451:17-22. 2006
    ..As usual the increase in the number of enzyme-catalyzed reactions for which apparent equilibrium constants can be calculated is larger than the number of reactions required to obtain the thermodynamic properties of the species involved...
  46. ncbi Low carbamoyl phosphate pools may drive Lactobacillus plantarum CO2-dependent growth phenotype
    Françoise Bringel
    UMR 7156 Université Louis Pasteur, CNRS Génétique Moléculaire, Genomique, Microbiologie, Département Microorganismes, Génomes, Environnement, Strasbourg, France
    J Mol Microbiol Biotechnol 14:22-30. 2008
    ..plantarum isolates...
  47. ncbi Protein expression under uracil privation in Lactobacillus sakei
    A Marceau
    , INRA, Domaine de Vilvert, 78350 Jouy en Josas, France
    FEMS Microbiol Lett 200:49-52. 2001
    ..Two of these polypeptides were identified as orotate phosphoribosyl transferase, catalyzing the fifth step of pyrimidine biosynthesis, and PyrR, the transcriptional regulator of the pyr operon, respectively...
  48. ncbi An engineered blockage within the ammonia tunnel of carbamoyl phosphate synthetase prevents the use of glutamine as a substrate but not ammonia
    X Huang
    Department of Chemistry, Texas A and M University, College Station, Texas 77843, USA
    Biochemistry 39:3240-7. 2000
    ..These results suggest the existence of an alternate route to the bicarbonate phosphorylation site when ammonia is provided as an external nitrogen source...
  49. pmc Glutamine-dependent carbamoyl-phosphate synthetase and other enzyme activities related to the pyrimidine pathway in spleen of Squalus acanthias (spiny dogfish)
    P M Anderson
    Department of Biochemistry, University of Minnesota, Duluth 55812
    Biochem J 261:523-9. 1989
    ..Biol. Chem. 262, 198-202] that dogfish express two different glutamine synthetases, and (3) provide indirect evidence that glutamine may not be available in liver for biosynthetic reactions other than urea formation...
  50. ncbi Urea cycle of Fasciola gigantica: purification and characterization of arginase
    Saleh A Mohamed
    Molecular Biology Department, National Research Centre, Cairo, Egypt
    Comp Biochem Physiol B Biochem Mol Biol 142:308-16. 2005
    ..The enzyme was activated with Mn2+, where the other metals Fe2+, Ca2+, Hg2+, Ni2+, Co2+ and Mg2+ had inhibitory effects...
  51. pmc Altered enzyme activities and citrulline synthesis in liver mitochondria from ornithine carbamoyltransferase-deficient sparse-furash mice
    N S Cohen
    Department of Biochemistry, University of Southern California School of Medicine, Los Angeles 90033
    Biochem J 257:251-7. 1989
    ..In addition, they clarify the role of ornithine transport and of protein-protein interactions in citrulline synthesis in normal mitochondria...
  52. ncbi African sharptooth catfish Clarias gariepinus does not detoxify ammonia to urea or amino acids but actively excretes ammonia during exposure to environmental ammonia
    Yuen K Ip
    Department of Biological Sciences, National University of Singapore, 10 Kent Ridge Road, Singapore 117543, Republic of Singapore
    Physiol Biochem Zool 77:242-54. 2004
    ..These results represent the first report on a freshwater fish (C. gariepinus) adopting active excretion of ammonia (probably NH4+) as a major strategy to defend against ammonia toxicity when exposed to environmental ammonia...
  53. ncbi Cloning of cDNA coding for carbamyl phosphate synthetase I and changes in levels of CPS1 mRNA during hepatocarcinogenesis
    S J Wu
    Faculty of Basic Medical Sciences, Peking Union Medical College, Beijing
    Sci Sin B 31:197-203. 1988
    cDNA coding for carbamyl phosphate synthetase I was cloned from recombinant plasmid with insert complementary to the mRNA for CPS1 followed by hybrid-selected translation screening...
  54. pmc On the origin of biochemistry at an alkaline hydrothermal vent
    William Martin
    Institute of Botany, University of Dusseldorf, 40225 Dusseldorf, Germany
    Philos Trans R Soc Lond B Biol Sci 362:1887-925. 2007
    ....
  55. doi Y-box binding protein-1 down-regulates expression of carbamoyl phosphate synthetase-I by suppressing CCAAT enhancer-binding protein-alpha function in mice
    Yen Rong Chen
    Institute of Molecular and Cellular Biosciences, The University of Tokyo, Japan
    Gastroenterology 137:330-40. 2009
    ..Despite expression of C/EBPalpha, CPS1 is not expressed in fetal liver, indicating an additional factor is involved in the regulation of CPS1 expression. The aim of this study was to elucidate the mechanism of CPS1 expression...
  56. ncbi Effects of short-chain acyl-CoA dehydrogenase deficiency on development expression of metabolic enzyme genes in the mouse
    M E Hinsdale
    Department of Comparative Medicine, School of Medicine, University of Alabama at Birmingham 35294, USA
    Biochem Mol Med 57:106-15. 1996
    ..SCAD) acyl-CoA dehydrogenases, pyruvate carboxylase (PC), phosphoenolpyruvate carboxykinase (PEPCK), carbamyl phosphate synthetase I (CPS), ornithine transcarbamylase (OTC), and argininosuccinate synthetase (AS) in fed or fasted ..
  57. ncbi N2-hydroxyguanosine 5'-monophosphate is a time-dependent inhibitor of Escherichia coli guanosine monophosphate synthetase
    M L Deras
    Department of Medicinal Chemistry and Molecular Pharmacology, Purdue University, West Lafayette, Indiana 47907 1333, USA
    Biochemistry 38:303-10. 1999
    ..In addition, these results complement recent structural studies of GMPS and implicate the formation of the XMP-adenylate intermediate inducing a probable conformational change that stimulates the hydrolysis of glutamine...
  58. pmc Development and evaluation of different normalization strategies for gene expression studies in Candida albicans biofilms by real-time PCR
    Heleen Nailis
    Laboratory for Pharmaceutical Microbiology, Ghent University, Harelbekestraat 72, B 9000, Ghent, Belgium
    BMC Mol Biol 7:25. 2006
    ..To validate our normalization strategies we determined differences in ALS1 and ALS3 expression levels between C. albicans biofilm cells and their planktonic counterparts...
  59. ncbi Uridine and cytidine nucleotide synthesis in renal hypertrophy: biochemical differences in response to the growth stimulus of diabetes and unilateral nephrectomy
    S Kunjara
    Department of Biochemistry, University College and Middlesex School of Medicine, London, Great Britain
    Biochem Med Metab Biol 47:168-80. 1992
    ....
  60. ncbi The dogfish shark (Squalus acanthias) increases both hepatic and extrahepatic ornithine urea cycle enzyme activities for nitrogen conservation after feeding
    Makiko Kajimura
    Department of Biology, McMaster University, Hamilton, Ontario, Canada
    Physiol Biochem Zool 79:602-13. 2006
    ..Furthermore, exogenous nitrogen from food is converted into urea not only by the liver but also by the muscle and to a small extent by the intestine...
  61. ncbi Ornithine transcarbamylase deficiency: a urea cycle defect
    Neil Gordon
    Wilmslow, UK
    Eur J Paediatr Neurol 7:115-21. 2003
    ..Liver transplant can be considered when symptoms are life-threatening, although there may be severe complications.Gene replacement therapy is the hope of the future...
  62. ncbi Orotic acid excretion and arginine metabolism
    Margaret E Brosnan
    Department of Biochemistry, Memorial University of Newfoundland, St John s, NL, Canada A1B 3X9
    J Nutr 137:1656S-1661S. 2007
    ....
  63. ncbi Molecular biology and biochemistry of malarial parasite pyrimidine biosynthetic pathway
    Jerapan Krungkrai
    Department of Biochemistry, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
    Southeast Asian J Trop Med Public Health 34:32-43. 2003
    ..The genes encoding these enzymes were identified in order, from the first to the sixth step, as CPSII (carbamyl phosphate synthase II), ATC (aspartate transcarbamylase), DHO (dihydroorotase), DHOD (dihydroorotate dehydrogenase, ..
  64. ncbi Neonatal pulmonary hypertension--urea-cycle intermediates, nitric oxide production, and carbamoyl-phosphate synthetase function
    D L Pearson
    Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    N Engl J Med 344:1832-8. 2001
    ....
  65. ncbi Effects of assembly and mutations outside the active site on the functional pH dependence of Escherichia coli aspartate transcarbamylase
    X Yuan
    Department of Biochemistry, University of Minnesota, St Paul 55108, USA
    J Biol Chem 271:1285-94. 1996
    ..productive binding of L-Asp to the binary complexes of the catalytic subunit (c3) and holoenzyme (c6r6) with carbamyl phosphate are different...
  66. ncbi [A case of late-onset carbamoyl phosphate synthetase I deficiency, presenting periodic psychotic episodes coinciding with menstrual periods]
    Y Wakutani
    Department of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University
    Rinsho Shinkeigaku 41:780-5. 2001
    ..Genomic DNA analysis showed that she was heterozygous in V1149G mutation. The mutation allele was derived from her father. The causative factor for absence or very low level of maternal CPSI mRNA will require further analysis...
  67. ncbi Relationship between carbamoyl-phosphate synthetase genotype and systemic vascular function
    Marshall L Summar
    Department of Pediatrics, Division of Medical Genetics, Vanderbilt University Medical Center, Nashville, Tenn 37232 6602, USA
    Hypertension 43:186-91. 2004
    ..943). These data indicate that a polymorphism in the gene encoding carbamoyl-phosphate synthetase 1 influences nitric oxide production as well as vascular smooth muscle reactivity...
  68. pmc Expression, purification, crystallization and preliminary X-ray crystallographic studies of a cold-adapted aspartate carbamoyltransferase from Moritella profunda
    Dirk De Vos
    Laboratorium voor Eiwitbiochemie en Eiwitengineering, Universiteit Gent, K L Ledeganckstraat 35, Ghent, Belgium
    Acta Crystallogr Sect F Struct Biol Cryst Commun 61:279-81. 2005
    ..The three-dimensional structure of the Escherichia coli ATCase was sufficient to solve the structure of the M. profunda ATCase via the molecular-replacement method and to obtain electron density of good quality...
  69. ncbi Differentially expressed genes associated with hepatitis B virus HBx and MHBs protein function in hepatocellular carcinoma
    Dae Ghon Kim
    Division of GI and Hepatology, Department of Internal Medicine, Chonbuk National University Medical School and Hospital, Jeonbuk, Republic of Korea
    Methods Mol Biol 317:141-55. 2006
    ..secretory trypsin inhibitor (PSTI), H19, guanidine nucleotide-binding protein alpha-1 subunit (GNAZ), carbamyl phosphate synthetase I (CPS I), insulin-like growth factor (IGF)-II, and 10 ribosomal proteins genes...
  70. ncbi Ornithine carbamoyltransferase from the extreme thermophile Thermus thermophilus--analysis of the gene and characterisation of the protein
    R Sanchez
    Laboratorium voor Erfelijkheidsleer en Microbiologie, Vrije Universiteit Brussel, Brussels, Belgium
    Eur J Biochem 248:466-74. 1997
    ..In the presence of its substrates the half-life time was several hours at 85 degrees C. Ionic and hydrophobic interactions contribute to the stability. The expression of T. thermophilus OTC was negatively regulated by arginine...
  71. ncbi Ornithine transcarbamylase and arginase I deficiency are responsible for diminished urea cycle function in the human hepatoblastoma cell line HepG2
    Demetra Mavri-Damelin
    The UCL Institute of Hepatology, Hampstead Campus, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Int J Biochem Cell Biol 39:555-64. 2007
    ..This strategy could provide a cell source capable of urea synthesis, and hence ammonia detoxificatory function, which would be useful in a bio-artificial liver...
  72. ncbi Lamprey parasitism of sharks and teleosts: high capacity urea excretion in an extant vertebrate relic
    Michael P Wilkie
    Department of Biology, Mount Allison University, Sackville, NB, Canada E4L 1E4
    Comp Biochem Physiol A Mol Integr Physiol 138:485-92. 2004
    ....
  73. ncbi Cell cycle-dependent regulation of pyrimidine biosynthesis
    Frederic D Sigoillot
    Department of Biochemistry and Molecular Biology, Wayne State University School of Medicine, Detroit, Michigan 48201, USA
    J Biol Chem 278:3403-9. 2003
    ..Thus, the cell cycle-dependent regulation of pyrimidine biosynthesis results from the sequential phosphorylation and dephosphorylation of CAD under the control of two important signaling cascades...
  74. ncbi Integration host factor (IHF) modulates the expression of the pyrimidine-specific promoter of the carAB operons of Escherichia coli K12 and Salmonella typhimurium LT2
    D Charlier
    Research Institute, CERIA COOVI, Brussels, Belgium
    Mol Gen Genet 237:273-86. 1993
    ..IHF thus appears to be required for maximal expression as well as for establishment of full repression. IHF could exert this function by modulating the binding of a pyrimidine-specific regulatory molecule...
  75. doi Urea cycle enzymes through the development of pacu (Piaractus mesopotamicus): the role of ornithine carbamoyl transferase
    Paulo Sérgio Monzani
    Laboratory of Adaptive Biochemistry, Department of Genetics and Evolution, Federal University of Sao Carlos, Rod Washington Luiz Km 235, CP 676, CEP 13565 905, Sao Carlos, SP, Brazil
    Fish Physiol Biochem 34:139-49. 2008
    ..We suppose that OCT during the embryogenesis is a control step regulating the cellular concentration of ornithine for polyamines synthesis...
  76. pmc Role of the glutamate dehydrogenase reaction in furnishing aspartate nitrogen for urea synthesis: studies in perfused rat liver with 15N
    Itzhak Nissim
    Division of Child Development and Rehabilitation Medicine, Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Biochem J 376:179-88. 2003
    ..In addition, the current findings suggest that the formation of aspartate via the mitochondrial aspartate aminotransferase reaction may play an important role in the synthesis of cytosolic argininosuccinate...
  77. doi Population screening in a Druze community: the challenge and the reward
    Tzipora C Falik-Zaccai
    Institute of Human Genetics, Western Galilee Hospital Nahariya, Israel
    Genet Med 10:903-9. 2008
    ..of metabolism cerebrotendinous xanthomatosis, prolidase deficiency, argininosuccinate lyase deficiency, and carbamyl phosphate synthetase I deficiency were identified in an isolated Druze village in northern Israel...
  78. ncbi Effect of alkalinity (pH 10) on ureogenesis in the air-breathing walking catfish, Clarias batrachus
    Nirmalendu Saha
    Biochemical Adaptation Laboratory, Department of Zoology, North Eastern Hill University, Shillong 93 022, India
    Comp Biochem Physiol A Mol Integr Physiol 132:353-64. 2002
    ..The activity of glutamine synthetase and three enzymes operating in the urea cycle (carbamyl phosphate synthetase, argininosuccinate synthetase, argininosuccinate lyase) increased significantly in hepatic and ..
  79. ncbi Growth factor-regulated expression of enzymes involved in nucleotide biosynthesis: a novel mechanism of growth factor action
    M G Gassmann
    Max Planck Institute of Biochemistry, Martinsried, Germany
    Oncogene 18:6667-76. 1999
    ....
  80. ncbi Role of ureogenesis in the mud-dwelled Singhi catfish (Heteropneustes fossilis) under condition of water shortage
    N Saha
    Biochemical Adaptation Laboratory, Department of Zoology, North Eastern Hill University, 793022, Shillong, India
    Comp Biochem Physiol A Mol Integr Physiol 128:137-46. 2001
    ..The activities (units/g tissue and /mg protein) of glutamine synthetase and three enzymes of the urea cycle, carbamyl phosphate synthetase, argininosuccinate synthetase and argininosuccinate lyase increased significantly in most of the ..
  81. ncbi Organization of carbamoyl-phosphate synthase genes in Neisseria gonorrhoeae includes a large, variable intergenic sequence which is also present in other Neisseria species
    F S Lawson
    Department of Biology, University of Ottawa, Ontario, Canada
    Microbiology 141:1183-91. 1995
    ..meningitidis and 18 commensal Neisseria isolates comprising nine species. This unexpected organization of the CPS genes in N. gonorrhoeae is therefore widespread throughout the genus Neisseria...
  82. ncbi Structure and catalytic mechanism of a novel N-succinyl-L-ornithine transcarbamylase in arginine biosynthesis of Bacteroides fragilis
    Dashuang Shi
    Children s Research Institute, Children s National Medical Center, George Washington University, Washington, D C 20010, USA
    J Biol Chem 281:20623-31. 2006
    ..Crystal structures of this novel transcarbamylase complexed with carbamyl phosphate and N-succinyl-L-norvaline, as well as sulfate and N-succinyl-L-norvaline have been determined and refined ..
  83. ncbi A comparison of the effects of environmental ammonia exposure on the Asian freshwater stingray Himantura signifer and the Amazonian freshwater stingray Potamotrygon motoro
    Yuen K Ip
    Department of Biological Science, National University of Singapore, Kent Ridge, Singapore 117543, Republic of Singapore
    J Exp Biol 206:3625-33. 2003
    ..Our results suggest that such a strategy began to develop in those elasmobranchs, e.g. H. signifer, that migrate into a freshwater environment from the sea but not in those permanently adapted to a freshwater environment...
  84. pmc Genetic variation in the mitochondrial enzyme carbamyl-phosphate synthetase I predisposes children to increased pulmonary artery pressure following surgical repair of congenital heart defects: a validated genetic association study
    Jeffrey A Canter
    Center for Human Genetics Research, Department of Molecular Physiology and Biophysics, Vanderbilt University Medical Center, Nashville, TN, USA
    Mitochondrion 7:204-10. 2007
    ..In conclusion, the CPSI T1405N genotype appears to be an important new factor in predicting susceptibility to increased PAP following surgical repair of congenital cardiac defects in children...
  85. doi Effects of crowding on ornithine-urea cycle enzyme mRNA expression and activity in gulf toadfish (Opsanus beta)
    Tammy Laberge
    Division of Marine Biology and Fisheries, Rosenstiel School of Marine and Atmospheric Science, University of Miami, 4600 Rickenbacker Causeway, Miami, FL 33149, USA
    J Exp Biol 212:2394-402. 2009
    ..These results suggest that the activities of O-UC enzymes are better predictors for urea production than O-UC enzyme mRNA expression levels...
  86. ncbi Urea synthesis in the African lungfish Protopterus dolloi--hepatic carbamoyl phosphate synthetase III and glutamine synthetase are upregulated by 6 days of aerial exposure
    Shit F Chew
    Natural Sciences, National Institute of Education, Nanyang Technological University, 1 Nanyang Walk, Singapore 637616, Republic of Singapore
    J Exp Biol 206:3615-24. 2003
    ..dolloi possesses transporters that facilitate the excretion of urea in water...
  87. ncbi Converting catabolic ornithine carbamoyltransferase to an anabolic enzyme
    H Baur
    Mikrobiologisches Institut, Eidgenossische Technische Hochschule, Zurich, Switzerland
    J Biol Chem 265:14728-31. 1990
    ..coli; the hybrid enzyme had a markedly reduced homotropic cooperativity. This gene fusion method should be generally useful for directed enzyme evolution...
  88. doi Structural insight on the control of urea synthesis: identification of the binding site for N-acetyl-L-glutamate, the essential allosteric activator of mitochondrial carbamoyl phosphate synthetase
    Satu Pekkala
    Centro de Investigacion Principe Felipe, Avda Autopista del Saler 16, Valencia 46012, Spain
    Biochem J 424:211-20. 2009
    ....
  89. ncbi The effects of various inhibitors on the regulation of orotic acid excretion in sparse-fur mutant mice (spf/Y) deficient in ornithine transcarbamylase
    J Nelson
    Department of Nutrition, University of Montreal, Quebec, Canada
    Chem Biol Interact 89:35-47. 1993
    ..The orotic aciduria remained insensitive to acivicin, indicating mitochondria as the source of carbamyl phosphate. However, orotate excretion was significantly decreased by PALA (P < 0...
  90. ncbi Site-directed mutagenesis of ATP binding residues of biotin carboxylase. Insight into the mechanism of catalysis
    V Sloane
    Division of Biochemistry and Molecular Biology, Louisiana State University, Baton Rouge, LA 70803, USA
    J Biol Chem 276:24991-6. 2001
    ..Included in this superfamily are d-Ala d-Ala ligase, glutathione synthetase, carbamyl phosphate synthetase, N(5)-carboxyaminoimidazole ribonucleotide synthetase, and glycinamide ribonucleotide ..
  91. pmc The use of nucleotide analogs to evaluate the mechanism of the heterotropic response of Escherichia coli aspartate transcarbamoylase
    J B Sakash
    Department of Chemistry, Merkert Chemistry Center, Boston College, Massachusetts 02467, USA
    Protein Sci 9:53-63. 2000
    ....
  92. pmc 3-isobutylmethylxanthine inhibits hepatic urea synthesis: protection by agmatine
    Itzhak Nissim
    Children s Hospital of Philadelphia, Division of Child Development, Department of Pediatrics, University of Pennsylvania School of Medicine, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    J Biol Chem 283:15063-71. 2008
    ..Together, the data may suggest a new experimental application of IBMX in studies of CPS-I malfunction and the use of agmatine as intervention therapy...
  93. doi Substrate binding and catalysis in carbamate kinase ascertained by crystallographic and site-directed mutagenesis studies: movements and significance of a unique globular subdomain of this key enzyme for fermentative ATP production in bacteria
    Santiago Ramon-Maiques
    Instituto de Biomedicina de Valencia Consejo Superior de Investigaciones Científicas and Centro de Investigación Biomédica en Red de Enfermedades Raras Instituto de Salud Carlos III, Jaime Roig 11, Valencia 46010, Spain
    J Mol Biol 397:1261-75. 2010
    ..It is concluded that the PSD movement allows CK to select against the abundant CP/carbamate analogues acetylphosphate/acetate and bicarbonate, rendering CK highly selective for CP/carbamate...
  94. ncbi Genomic organization of the human gene (CA5) and pseudogene for mitochondrial carbonic anhydrase V and their localization to chromosomes 16q and 16p
    Y Nagao
    Edward A Doisy Department of Biochemistry and Molecular Biology, St Louis University School of Medicine, Missouri 63104, USA
    Genomics 28:477-84. 1995
    ..FISH analysis allowed CA5 to be assigned to chromosome 16q24.3. An unprocessed pseudogene containing sequence homologous to exons 3-7 and introns 3-6 was also isolated and was assigned by FISH analysis to chromosome 16p11.2-p12...
  95. pmc A single mutation in the active site swaps the substrate specificity of N-acetyl-L-ornithine transcarbamylase and N-succinyl-L-ornithine transcarbamylase
    Dashuang Shi
    Children s National Medical Center, The George Washington University, Washington, DC 20010, USA
    Protein Sci 16:1689-99. 2007
    Transcarbamylases catalyze the transfer of the carbamyl group from carbamyl phosphate (CP) to an amino group of a second substrate such as aspartate, ornithine, or putrescine...
  96. pmc Metabolic enzymes from psychrophilic bacteria: challenge of adaptation to low temperatures in ornithine carbamoyltransferase from Moritella abyssi
    Ying Xu
    J M Wiame Research Institute, Microbiology, Free University of Brussels, B 1070 Brussels
    J Bacteriol 185:2161-8. 2003
    ..OTCase(Mab) adaptation to cold indicates a trade-off between affinity and catalytic velocity, suggesting that optimization of key metabolic enzymes at low temperatures may be constrained by natural limits...
  97. ncbi Shifting patterns of nitrogen excretion and amino acid catabolism capacity during the life cycle of the sea lamprey (Petromyzon marinus)
    Michael P Wilkie
    Department of Biology, Wilfrid Laurier University, Waterloo, Ontario, N2L 3C5, Canada
    Physiol Biochem Zool 79:885-98. 2006
    ..The subsequent generation of energy-rich carbon skeletons can then be oxidized or retained for glycogen and fatty acid synthesis, which are essential fuels for the upstream migratory and spawning phases of the sea lamprey's life cycle...
  98. pmc Adenovirus preterminal protein binds to the CAD enzyme at active sites of viral DNA replication on the nuclear matrix
    P C Angeletti
    Department of Biochemistry and Molecular Genetics, School of Medicine, University of Alabama at Birmingham, 35294 0005, USA
    J Virol 72:2896-904. 1998
    ..Amino acid sequencing of the 150-kDa band revealed sequence identity to carbamyl phosphate synthetase I (CPS I) and a high degree of homology to the related trifunctional enzyme known as CAD (for ..
  99. ncbi Nitrogen metabolism in the African lungfish (Protopterus dolloi) aestivating in a mucus cocoon on land
    Shit F Chew
    Natural Sciences, National Institute of Education, Nanyang Technological University, 1 Nanyang Walk, Singapore 637616, Republic of Singapore
    J Exp Biol 207:777-86. 2004
    ..Our results indicate that a reduction in ammonia production and decreases in hepatic arginine and cranial tryptophan contents are important facets of aestivation in P. dolloi...
  100. ncbi The amidotransferase family of enzymes: molecular machines for the production and delivery of ammonia
    F M Raushel
    Department of Chemistry, Texas A and M University, College Station 77843, USA
    Biochemistry 38:7891-9. 1999
    ..Molecular tunnels between distant active sites have also been identified in tryptophan synthase and glutamine phosphoribosyl pyrophosphate amidotransferase and are likely architectural features in an expanding list of enzymes...
  101. ncbi Opposite effects on regulation of urea synthesis by early and late uraemia in rats
    Susanne Schouw Nielsen
    Department of Medicine V Hepatology and Gastroenterology, Aarhus University Hospital, 44 Noerrebrogade, DK 8000 Aarhus C, Denmark
    Clin Nutr 26:245-51. 2007
    ..The aims were to investigate the effects of early and late experimental renal failure on the regulation of hepatic urea synthesis and the expression of urea cycle enzyme genes in the liver...

Research Grants76

  1. Genotype Analysis for Diagnosis of Urea Cycle Disorders
    Steven Dobrowolski; Fiscal Year: 2007
    ..Products of the following genes are required for ureagenesis: N-acetylglutamate synthetase carbamyl phosphate synthetase 1 , ornithine transcarbamylase , argininosuccinate synthetase , argininosuccinate lyase , ..
  2. REGULATION OF 15N UREA ISOTOPOMERS PRODUCTION
    Itzhak Nissim; Fiscal Year: 2010
    ..Our preliminary results suggest that such treatment is feasible. The outcome of the propose study may provide new guidelines for evaluation, diagnosis and treatment of defective urea synthesis in NAFLD or MS. ..
  3. REGULATION OF 15N UREA ISOTOPOMERS PRODUCTION
    Itzhak Nissim; Fiscal Year: 2010
    ..Our preliminary results suggest that such treatment is feasible. The outcome of the propose study may provide new guidelines for evaluation, diagnosis and treatment of defective urea synthesis in NAFLD or MS. ..
  4. STRUCTURE AND FUNCTION OF CARBAMYL PHOSPHATE SYNTHETASE
    CAROL LUSTY; Fiscal Year: 1993
    The synthesis of carbamyl phosphate, an intermediate in pyrimidine and arginine biosynthesis, is catalyzed by ammonia and glutamine dependent carbamyl phosphate synthetase (CPS)...
  5. MECHANISMS OF THYROID HORMONE ACTION IN DEVELOPMENT
    VALERIE GALTON; Fiscal Year: 1991
    ..The levels of translatable mRNA for carbamyl phosphate synthetase (CPS) present in liver during spontaneous and induced metamorphosis will be determined and ..
  6. COMMON POLYMORPHISM EFFECTS ON UREA CYCLE FUNCTION
    Marshall Summar; Fiscal Year: 2001
    ..My research has focused on characterizing the gene encoding the first, rate-limiting step of ureagenesis, carbamyl phosphate synthetase I (CPSI)...
  7. Nitric Oxide Precursors and Congenital Heart Surgery
    Frederick Barr; Fiscal Year: 2007
    ..Finally, a genetic single nucleotide polymorphism (SNP) in the rate limiting urea cycle enzyme, carbamyl phosphate synthetase I (CPSl T1405N), appeared to affect postoperative plasma arginine levels and pulmonary vascular ..
  8. N-carbamylglutamate in the treatment of hyperammonemia
    Mendel Tuchman; Fiscal Year: 2009
    ..that cause hyperammonemia and consequent brain damage: N-acetylglutamate synthase (NAGS) deficiency, carbamyl phosphate synthetase I (CPSI) deficiency, propionic acidemia (PA), methylmalonic acidemia (MMA) and hyperinsulinism ..
  9. REGULATION OF 15N UREA ISOTOPOMERS PRODUCTION
    Itzhak Nissim; Fiscal Year: 2007
    ..The proposed studies are of clinical as well as scientific significance. The data to be generated may have tremendous clinical impact by advancing the development of a protocol to ameliorate impaired urea synthesis. ..
  10. REGULATION OF 15N UREA ISOTOPOMERS PRODUCTION
    Itzhak Nissim; Fiscal Year: 2002
    ..A long term goal is to apply our experimental approach to human subjects in cases such as sepsis, hepatic encephalopathy and/or perturbed hormonal status, such as in diabetes. ..
  11. GENE STRUCTURE OF CARBAMYL PHOSPHATE SYNTHETASE
    CAROL LUSTY; Fiscal Year: 1992
    b>Carbamyl phosphate synthetase is an important housekeeping enzyme that provides carbamyl phosphate for arginine and pyrimidine biosynthesis. Our previous studies on the carbamyl phosphate synthetase genes of E...
  12. Structural Studies of the Pyrimidine Pathway
    Jacqueline Vitali; Fiscal Year: 2004
    ..Finally, understanding the means of extreme heat stability will allow us to engineer selected features in other aspartate transcarbamoylases to obtain enzymes with enhanced thermostability. ..
  13. CONTROL OF PYRIMIDINE BIOSYNTHESIS IN MAMMALIAN CELLS
    David Evans; Fiscal Year: 1993
    ..The first three enzymes of the pathway, carbamyl phosphate synthetase, aspartate transcarbamylase and dihydroorotase are carried by a single 230 Kd polypeptide chain...
  14. T. Gondii: Pyrimidine Synthesis as a Chemo Target
    David J Bzik; Fiscal Year: 2010
    ..Consequently, this research project will accelerate the discovery of new vaccines and treatments for devastating opportunistic parasite infections in AIDS. ..
  15. Structure-Function Analysis of Enzymes
    Hazel Holden; Fiscal Year: 2004
    ....