ASS1

Summary

Gene Symbol: ASS1
Description: argininosuccinate synthase 1
Alias: ASS, CTLN1, argininosuccinate synthase, argininosuccinate synthetase 1, citrulline--aspartate ligase, citrulline-aspartate ligase
Species: human

Top Publications

  1. ncbi Correction of argininosuccinate synthetase (AS) deficiency in a murine model of citrullinemia with recombinant adenovirus carrying human AS cDNA
    X Ye
    Children s Research Institute, Children s National Medical Center, Washington, DC 20010, USA
    Gene Ther 7:1777-82. 2000
  2. ncbi Mild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1)
    Johannes Haberle
    Universitatsklinikum Munster, Klinik und Poliklinik für Kinderheilkunde, Albert Schweitzer Strasse 33, D 48149 Munster, Germany
    Mol Genet Metab 80:302-6. 2003
  3. pmc Slc25a13-knockout mice harbor metabolic deficits but fail to display hallmarks of adult-onset type II citrullinemia
    David S Sinasac
    Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8
    Mol Cell Biol 24:527-36. 2004
  4. ncbi [Adult-onset citrullinemia]
    Shu Ichi Ikeda
    Department of Internal Medicine Neurology and Rheumatology, Shinshu University School of Medicine, Matsumoto, Japan
    Brain Nerve 59:59-66. 2007
  5. ncbi Identification of two novel mutations in the SLC25A13 gene and detection of seven mutations in 102 patients with adult-onset type II citrullinemia
    T Yasuda
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Japan
    Hum Genet 107:537-45. 2000
  6. ncbi [Adult-onset citrullinemia]
    Shu Ichi Ikeda
    Department of Internal Medicine Neurology and Rheumatology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
    No To Shinkei 59:59-66. 2007
  7. ncbi Genomic structure of the adult-onset type II citrullinemia gene, SLC25A13, and cloning and expression of its mouse homologue
    D S Sinasac
    Department of Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario, Canada
    Genomics 62:289-92. 1999
  8. ncbi Generation of a mouse model for citrullinemia by targeted disruption of the argininosuccinate synthetase gene
    G Patejunas
    Institute for Molecular Genetics, Baylor College of Medicine, Houston, Texas 77030
    Somat Cell Mol Genet 20:55-60. 1994
  9. ncbi Arginine metabolism and the synthesis of nitric oxide in the nervous system
    H Wiesinger
    Physiologisch chemisches Institut der Universität, Hoppe Seyler Strasse 4, D 72076, Tubingen, Germany
    Prog Neurobiol 64:365-91. 2001
  10. ncbi Pregnancy in a healthy woman with untreated citrullinemia
    Murray A Potter
    Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada
    Am J Med Genet A 129:77-82. 2004

Research Grants

Detail Information

Publications167 found, 100 shown here

  1. ncbi Correction of argininosuccinate synthetase (AS) deficiency in a murine model of citrullinemia with recombinant adenovirus carrying human AS cDNA
    X Ye
    Children s Research Institute, Children s National Medical Center, Washington, DC 20010, USA
    Gene Ther 7:1777-82. 2000
    ..We have explored the use of adenoviral vectors as a treatment modality in a murine model of citrullinemia, the Ass mouse. The Ass mouse has no endogenous AS activity due to a targeted interruption of the AS gene...
  2. ncbi Mild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1)
    Johannes Haberle
    Universitatsklinikum Munster, Klinik und Poliklinik für Kinderheilkunde, Albert Schweitzer Strasse 33, D 48149 Munster, Germany
    Mol Genet Metab 80:302-6. 2003
    Citrullinemia is caused by either deficiency of argininosuccinate synthetase (ASS, citrullinemia type 1) or a defect of the SLC25A13 gene encoding a mitochondrial aspartate-glutamate transporter (citrullinemia type II)...
  3. pmc Slc25a13-knockout mice harbor metabolic deficits but fail to display hallmarks of adult-onset type II citrullinemia
    David S Sinasac
    Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8
    Mol Cell Biol 24:527-36. 2004
    ..The absence of citrin leads to a liver-specific, quantitative decrease of argininosuccinate synthetase (ASS), causing hyperammonemia and citrullinemia...
  4. ncbi [Adult-onset citrullinemia]
    Shu Ichi Ikeda
    Department of Internal Medicine Neurology and Rheumatology, Shinshu University School of Medicine, Matsumoto, Japan
    Brain Nerve 59:59-66. 2007
    ..of citrulline and ammonia in the plasma, which is ascribed to a deficiency of argininosuccinate synthetase (ASS), one of the urea cycle enzymes mainly located in the liver...
  5. ncbi Identification of two novel mutations in the SLC25A13 gene and detection of seven mutations in 102 patients with adult-onset type II citrullinemia
    T Yasuda
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Japan
    Hum Genet 107:537-45. 2000
    ..type II citrullinemia (CTLN2) is characterized by a liver-specific deficiency of argininosuccinate synthetase (ASS) protein. We have recently identified the gene responsible for CTLN2, viz...
  6. ncbi [Adult-onset citrullinemia]
    Shu Ichi Ikeda
    Department of Internal Medicine Neurology and Rheumatology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
    No To Shinkei 59:59-66. 2007
    ..of citrulline and ammonia in the plasma, which is ascribed to a deficiency of argininosuccinate synthetase (ASS), one of the urea cycle enzymes mainly located in the liver...
  7. ncbi Genomic structure of the adult-onset type II citrullinemia gene, SLC25A13, and cloning and expression of its mouse homologue
    D S Sinasac
    Department of Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario, Canada
    Genomics 62:289-92. 1999
    Citrullinemia is an autosomal recessive disease characterized by an argininosuccinate synthetase (ASS) deficiency...
  8. ncbi Generation of a mouse model for citrullinemia by targeted disruption of the argininosuccinate synthetase gene
    G Patejunas
    Institute for Molecular Genetics, Baylor College of Medicine, Houston, Texas 77030
    Somat Cell Mol Genet 20:55-60. 1994
    Argininosuccinate synthetase (ASS) is a urea cycle enzyme that forms argininosuccinate from citrulline and aspartate. Mutations at the ASS locus in man cause the inherited disease, citrullinemia...
  9. ncbi Arginine metabolism and the synthesis of nitric oxide in the nervous system
    H Wiesinger
    Physiologisch chemisches Institut der Universität, Hoppe Seyler Strasse 4, D 72076, Tubingen, Germany
    Prog Neurobiol 64:365-91. 2001
    ..Argininosuccinate synthetase (ASS) and argininosuccinate lyase which together can recycle the NOS coproduct L-citrulline to L-arginine are expressed ..
  10. ncbi Pregnancy in a healthy woman with untreated citrullinemia
    Murray A Potter
    Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada
    Am J Med Genet A 129:77-82. 2004
    ..on a second successful pregnancy in a woman with citrullinemia due to argininosuccinate synthetase deficiency (CTLN1)...
  11. ncbi Role of the L-citrulline/L-arginine cycle in iNANC nerve-mediated nitric oxide production and airway smooth muscle relaxation in allergic asthma
    Harm Maarsingh
    Department of Molecular Pharmacology, University Centre for Pharmacy, A Deusinglaan 1, 9713 AV Groningen, The Netherlands
    Eur J Pharmacol 546:171-6. 2006
    ..In NO-producing cells, L-citrulline can be recycled to L-arginine in a two-step reaction involving argininosuccinate synthase (ASS) and -lyase (ASL)...
  12. ncbi Mutation analysis of Korean patients with citrullinemia
    K M Hong
    Department of Biochemistry, Wonkwang University College of Medicine, Iksan, Korea
    Mol Cells 10:465-8. 2000
    Citrullinemia is an autosomal recessive disease due to the mutations in the argininosuccinate synthetase (ASS) gene. Mutation analysis was performed on three Korean patients with citrullinemia...
  13. ncbi Almost all about citrulline in mammals
    E Curis
    Laboratoire de Biomathématiques, E A 2498, Faculte de Pharmacie, Universite Rene Descartes, Paris, France
    Amino Acids 29:177-205. 2005
    ..synthase (NOS) and ornithine carbamoyltransferase (OCT) which produce citrulline, and argininosuccinate synthetase (ASS) that converts it into argininosuccinate...
  14. doi Assessment of the potential for displacement interactions with sugammadex: a pharmacokinetic-pharmacodynamic modelling approach
    Alex Zwiers
    Schering Plough Research Institute, a Division of MSD, Newhouse, Scotland, UK
    Clin Drug Investig 31:101-11. 2011
    ..Cyclodextrins are known to form inclusion complexes with other compounds...
  15. ncbi The evolution of aging: a new approach to an old problem of biology
    J T Bowles
    Med Hypotheses 51:179-221. 1998
    ..This advantage of aging led to the evolution of aging systems (ASs). Periodic predation was the most prevalent episodic selection pressure in evolution...
  16. ncbi Effects of acute ammonia toxicity on nitric oxide (NO), citrulline-NO cycle enzymes, arginase and related metabolites in different regions of rat brain
    M Swamy
    Department of Chemical Pathology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia
    Neurosci Res 53:116-22. 2005
    ..by-product of the NOS reaction, can be recycled to arginine by successive actions of argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL) via the citrulline-NO cycle...
  17. ncbi Argininosuccinate synthetase and argininosuccinate lyase: two ornithine cycle enzymes from Agaricus bisporus
    Matthijs J M Wagemaker
    Department of Microbiology, IWWR, Radboud University Nijmegen, Toernooiveld 1, NL 6525 ED Nijmegen, The Netherlands
    Mycol Res 111:493-502. 2007
    ..Argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL) are two ornithine cycle enzymes catalysing the last two steps in the arginine ..
  18. ncbi Alterations in glomerular anionic sites in canine anti-glomerular basement membrane nephritis with onset of severe proteinuria
    J Sugimoto
    Mitsubishi Kasei Corporation, Research Center, School of Veterinary Medicine, Azabu University, Yokohama, Japan
    Toxicol Pathol 22:316-23. 1994
    ..Alterations of anionic sites (ASs) of glomerular basement membrane (GBM) in peripheral, proximal, and paramesangial portions were studied ..
  19. doi Brain nitric oxide production by a proline-rich decapeptide from Bothrops jararaca venom improves baroreflex sensitivity of spontaneously hypertensive rats
    Claudiana Lameu
    Departamento de Bioquimica, Instituto de Quimica, Universidade de Sao Paulo, Sao Paulo, Brazil
    Hypertens Res 33:1283-8. 2010
    ..of nitric oxide (NO), which is synthesized by NO synthase (NOS) in the citrulline-NO cycle (with argininosuccinate synthase (ASS) activity being the rate-limiting step), contributes to impaired baroreflex...
  20. pmc Argininosuccinate synthetase is a functional target for a snake venom anti-hypertensive peptide: role in arginine and nitric oxide production
    Juliano R Guerreiro
    Center for Applied Toxinology CAT CEPID, Instituto Butantan, SP 05503 900, Sao Paulo, Brazil
    J Biol Chem 284:20022-33. 2009
    ..for kidney cytosolic proteins with affinity for Bj-BPP-10c and demonstrate that argininosuccinate synthetase (AsS) is the major protein binding to the peptide...
  21. doi Reduced argininosuccinate synthetase is a predictive biomarker for the development of pulmonary metastasis in patients with osteosarcoma
    Eisuke Kobayashi
    Chemotherapy Division, National Cancer Centre Research Institute, Chuo Ku, Tokyo, Japan
    Mol Cancer Ther 9:535-44. 2010
    ..We identified argininosuccinate synthetase (ASS) as a gene differentially expressed with the highest statistical significance (Welch's t test, P = 2.2 x 10(-5))...
  22. doi Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defects
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Metabolism 58:278-82. 2009
    ..NOS reaction, can be recycled to arginine by the 2 enzymes acting in the urea cycle: argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL)...
  23. ncbi [Effects of high sodium intake on ventricular remodeling in mice]
    P V Ennezat
    INSERM U400,
    Arch Mal Coeur Vaiss 91:935-9. 1998
    ..6%: 0.64 +/- 0.01, 2%: 0.62 +/- 0.08 and 4%: 0.67 +/- 0.03 mm respectively) but LV septal wall thickness ass increased in a salt diet dependent manner (0.6%: 0.63 +/- 0.01, 2%: 0.75 +/- 0.01, 4%: 0.80 +/- 0.02 mm, p < 0...
  24. ncbi Identification and preliminary validation of novel biomarkers of acute hepatic ischaemia/reperfusion injury using dual-platform proteomic/degradomic approaches
    S I Svetlov
    Laboratory of Molecular and Cellular Mechanisims of Injury, University of Florida College of Medicine, Gainesville, FL 32610, USA
    Biomarkers 11:355-69. 2006
    ..liquid chromatography-tandem mass-spectrometry, we identified several hepatic proteins, including argininosuccinate synthase (ASS) and estrogen sulfotransferase (EST-1), which were degraded in the liver and rapidly released into ..
  25. ncbi Cosegregation of open-angle glaucoma and the nail-patella syndrome
    P R Lichter
    Department of Ophthalmology, University of Michigan, Ann Arbor 48105, USA
    Am J Ophthalmol 124:506-15. 1997
    ..To evaluate two families ascertained only for the presence of glaucoma in which both nail-patella syndrome and glaucoma occur in several generations and to determine whether the two diseases are genetically related...
  26. ncbi Activated microglia cells express argininosuccinate synthetase and argininosuccinate lyase in the rat brain after transient ischemia
    Elisa Bizzoco
    Department of Anatomy, Histology and Forensic Medicine, Section of Histology, University of Florence, Viale Pieraccini, 6, 50139 Florence, Italy
    Exp Neurol 208:100-9. 2007
    Argininosuccinate-synthetase (ASS), argininosuccinate-lyase (ASL) and nitric oxide synthase (NOS) act in the l-arginine-NO-l-citrulline cycle...
  27. doi Genetic variation in the urea cycle: a model resource for investigating key candidate genes for common diseases
    Sabrina Mitchell
    Center for Human Genetics Research, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA
    Hum Mutat 30:56-60. 2009
    ..cycle: carbamoyl-phosphate synthetase 1 (CPS1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS1), argininosuccinate lyase (ASL), and arginase 1 (ARG1)...
  28. ncbi Pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase
    Tawnya L Bowles
    Department of Surgery, University of California, Davis Medical Center, Sacramento, CA 95817, USA
    Int J Cancer 123:1950-5. 2008
    ..the non-essential amino acid arginine from aspartate and citrulline using the enzyme argininosuccinate synthetase (ASS)...
  29. ncbi Transient ischemia increases neuronal nitric oxide synthase, argininosuccinate synthetase and argininosuccinate lyase co-expression in rat striatal neurons
    Elisa Bizzoco
    Department of Anatomy, Histology and Forensic Medicine, Section of Histology, University of Florence, Viale G Pieraccini, 6, 50134 Florence, Italy
    Exp Neurol 204:252-9. 2007
    ..Argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL) and nNOS act in the L-arginine-NO-L-citrulline cycle permitting a correct NO ..
  30. 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
    ..Quantitative PCR was utilized to determine liver GS, CPS, OCT, ARG, argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL) mRNA expression...
  31. ncbi Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action
    M Tomomura
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Sakuragaoka, Japan
    FEBS Lett 399:310-2. 1996
    ..effects of fatty acids on urea cycle enzyme, carbamoylphosphate synthetase (CPS) and argininosuccinate synthetase (ASS), mRNA levels were examined in rat primary cultured hepatocytes...
  32. doi In vivo renal arginine release is impaired throughout development of chronic kidney disease
    Gin Fu Chen
    Department of Physiology and Functional Genomics, University of Florida, Gainesville, Florida 32610, USA
    Am J Physiol Renal Physiol 298:F95-102. 2010
    The kidney is a major site of arginine synthesis where citrulline is converted to arginine via argininosuccinate synthase (ASS) and lyase (ASL)...
  33. ncbi NADPH-diaphorase and cytosolic urea cycle enzymes in the rat spinal cord
    H Nakamura
    Department of Anatomy, Faculty of Medicine, Kyushu University, Fukuoka, Japan
    J Comp Neurol 385:616-26. 1997
    Nitric oxide synthase (NOS), argininosuccinate synthetase (ASS), and argininosuccinate lyase (ASL) compose a cyclic pathway to form nitric oxide (NO). These enzymes, however, are localized differentially in most regions of the brain...
  34. ncbi Activated microglia modulate astroglial enzymes involved in oxidative and inflammatory stress and increase the resistance of astrocytes to oxidative stress in vitro
    Claudia Röhl
    Department of Anatomy, University of Kiel, Olshausenstr 40, D 24098 Kiel, Germany
    Glia 56:1114-26. 2008
    ..dismutases (Sod), peroxiredoxins, glutathione S-transferases (Gst), nucleoside diphosphate kinase B, argininosuccinate synthase (Ass), and cellular retinol-binding protein I (Rbp1)...
  35. ncbi Citrulline-argininosuccinate-arginine cycle coupled to Ca2+-signaling in rat pancreatic beta-cells
    M Nakata
    Department of Laboratory Medicine, Faculty of Medicine, Kagoshima University, Japan
    Biochem Biophys Res Commun 235:619-24. 1997
    ..The enzymes involved in citrulline-arginine metabolism, argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), and NOS were expressed in rat islets and insulinoma HIT T15 cells...
  36. ncbi Pegylated recombinant human arginase (rhArg-peg5,000mw) inhibits the in vitro and in vivo proliferation of human hepatocellular carcinoma through arginine depletion
    Paul Ning Man Cheng
    Cancer Drug R and D Centre and Department of Applied Biology and Chemical Technology, The Hong Kong Polytechnic University, Hung Hom, Kowloon, Hong Kong, China
    Cancer Res 67:309-17. 2007
    ..HCC) is believed to be auxotrophic for arginine through the lack of expression of argininosuccinate synthetase (ASS)...
  37. doi Induction of arginosuccinate synthetase (ASS) expression affects the antiproliferative activity of arginine deiminase (ADI) in melanoma cells
    Antonella Manca
    Institute of Biomolecular Chemistry, National Research Council, Traversa La Crucca, 07100 Sassari, Italy
    Oncol Rep 25:1495-502. 2011
    ..of arginine is mainly dependent on activity of ornithine transcarbamylase (OTC) and argininosuccinate synthetase (ASS) enzymes. We evaluated the effect of ADI treatment on OTC and ASS expression in a series of melanoma cell lines...
  38. ncbi Alterations in glomerular anionic sites in the autologous phase of canine anti-glomerular basement membrane nephritis
    J Sugimoto
    Mitsubishi Chemical Corporation, Yokohama Research Center, Japan
    Toxicol Pathol 24:627-35. 1996
    ..An alteration of anionic sites (ASs) of GBM was studied quantitatively using polyethyleneimine as a cationic probe by electron microscopy at weeks 1, 2,..
  39. ncbi The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein
    K Kobayashi
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Japan
    Nat Genet 22:159-63. 1999
    ..CTLN2 differs from classical citrullinaemia (CTLN1, OMIM 215700) in that CTLN1 is neonatal or infantile in onset, with ASS enzyme defects (in all tissues) arising ..
  40. ncbi Glutamine and interleukin-1beta interact at the level of Sp1 and nuclear factor-kappaB to regulate argininosuccinate synthetase gene expression
    Carole Brasse-Lagnel
    Groupe Appareil Digestif, Environnement et Nutrition, Institut Fédératif de Recherches Multidisciplinaires sur Peptides, Universite de Rouen, France
    FEBS J 274:5250-62. 2007
    We previously demonstrated that the expression of the argininosuccinate synthetase (ASS) gene, a key step in nitric oxide production, is stimulated either by interleukin-1beta[Brasse-Lagnel et al...
  41. pmc Targeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes
    Macus Tien Kuo
    Department of Molecular Pathology, The University of Texas M D Anderson Cancer Center, Houston, USA
    Oncotarget 1:246-51. 2010
    ..carcinoma (HCC) are auxotrophic for arginine (Arg), because they do not express argininosuccinate synthetase (ASS), the rate-limiting enzyme for the biosynthesis of arginine from citrulline...
  42. pmc Arginine deiminase as a novel therapy for prostate cancer induces autophagy and caspase-independent apoptosis
    Randie H Kim
    Department of Biological Chemistry, Division of Surgical Oncology, Center for Biophotonics and Science Technology, University of California at Davis, Sacramento, California 95817, USA
    Cancer Res 69:700-8. 2009
    ..The efficacy of ADI-PEG20 is directly correlated with argininosuccinate synthetase (ASS) deficiency...
  43. doi Citrin deficiency, a perplexing global disorder
    David Dimmock
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, BCM225, Houston, TX 77030, USA
    Mol Genet Metab 96:44-9. 2009
    ..A key diagnostic feature has been the deficient argininosuccinate synthetase (ASS) activity (E.C. 6.3.4.5) in liver, with normal activity in skin fibroblasts...
  44. ncbi Inducible nitric oxide synthase and argininosuccinate synthetase: co-induction in brain tissue of patients with Alzheimer's dementia and following stimulation with beta-amyloid 1-42 in vitro
    Jürgen Haas
    Department of Neurology, University of Heidelberg, Heidelberg, Germany
    Neurosci Lett 322:121-5. 2002
    ..levels of inducible nitric oxide synthase (iNOS) and the arginine regenerating enzyme argininosuccinate synthetase (ASS) within cortices of AD patients suggesting high output NO production...
  45. ncbi Assignment of the structural gene for argininosuccinate synthetase to proximal mouse chromosome 2
    M J Jackson
    Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030
    Genomics 6:545-7. 1990
    In order to develop linkage markers for the murine argininosuccinate synthetase locus (Ass-1), we have searched for restriction fragment length polymorphisms in the mouse genome using cloned sequences from the mouse arginosuccinate ..
  46. doi Transient fulminant liver failure as an initial presentation in citrullinemia type I
    Hannaneh Faghfoury
    Division of Clinical and Metabolic Genetics, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada
    Mol Genet Metab 102:413-7. 2011
    Citrullinemia type I (CTLN1) is a urea cycle disorder which typically presents in the neonatal period or infancy with hyperammonemia and concurrent neurologic deterioration...
  47. ncbi Analysis of gene expression identifies differentially expressed genes and pathways associated with lymphatic dissemination in patients with adenocarcinoma of the esophagus
    S M Lagarde
    Department of Surgery, Academic Medical Center at the University of Amsterdam, Amsterdam, The Netherlands
    Ann Surg Oncol 15:3459-70. 2008
    ..The aim of this study was to discover a prognostic gene expression profile for lymphatic dissemination in EA and to identify genes and pathways that provide oncological insight in lymphatic dissemination...
  48. ncbi Genetic alterations determine chemotherapy resistance in childhood T-ALL: modelling in stage-specific cell lines and correlation with diagnostic patient samples
    David A Estes
    Department of Pathology, University of New Mexico Cancer Research and Treatment Center, Albuquerque, NM 87112, USA
    Br J Haematol 139:20-30. 2007
    ..Microarray analysis identified upregulation of asparagine synthetase (ASNS) and argininosuccinate synthase 1 (ASS1) to cell lines with acquired resistance to L-asp, and in the case of DNR, upregulation of ATP-..
  49. doi Recombinant human arginase inhibits proliferation of human hepatocellular carcinoma by inducing cell cycle arrest
    T L Lam
    Department of Applied Biology and Chemical Technology, The Hong Kong Polytechnic University, Hung Hom, Kowloon, Hong Kong, China
    Cancer Lett 277:91-100. 2009
    ..Expression profiling of argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL) and OTC in 40 HCC tumor biopsy specimens predicted that 16 of the patients would be ..
  50. pmc The role of molecular testing and enzyme analysis in the management of hypomorphic citrullinemia
    David P Dimmock
    Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA
    Am J Med Genet A 146:2885-90. 2008
    ..enzyme activity was not deficient in all patients with biallelic mutations suggesting this test cannot exclude the ASS1 locus in patients with mildly elevated plasma citrulline...
  51. doi Arginine deprivation and argininosuccinate synthetase expression in the treatment of cancer
    Barbara Delage
    Queen Mary University of London, Centre for Molecular Oncology and Imaging, Institute of Cancer and CR UK Clinical Centre, Barts and the London School of Medicine, Charterhouse Square, London, United Kingdom
    Int J Cancer 126:2762-72. 2010
    ..Tumoural downregulation of the enzyme argininosuccinate synthetase (ASS1), a recognised rate-limiting step in arginine synthesis, results in an intrinsic dependence on extracellular ..
  52. pmc Endothelial argininosuccinate synthetase 1 regulates nitric oxide production and monocyte adhesion under static and laminar shear stress conditions
    Gyeong In Mun
    Department of Molecular Medicine, Cell and Matrix Research Institute, BK21 Medical Education Program for Human Resources, Kyungpook National University School of Medicine, Daegu 700 422, Korea
    J Biol Chem 286:2536-42. 2011
    ..Recent studies demonstrated that LSS also increases the expression of argininosuccinate synthetase 1 (ASS1) that regulates the provision of L-arginine, the substrate of NOS3...
  53. ncbi ADI, autophagy and apoptosis: metabolic stress as a therapeutic option for prostate cancer
    Randie H Kim
    Department of Biological Chemistry, UC Davis Cancer Center, University of California Davis, 4645 2nd Avenue, Sacramento, CA 95817, USA
    Autophagy 5:567-8. 2009
    ..These cancers lose expression of an arginine synthesis enzyme, argininosuccinate synthetase (ASS), and are susceptible to arginine deprivation by arginine deiminase (ADI)...
  54. ncbi [Secondary prevention of ischemic strokes: effect of dosage of aspirin]
    J Alvarez-Sabin
    Servicio de Neurologia, Hospital General i Universitari Vall d Hebron, Barcelona, Espana
    Rev Neurol 25:541-4. 1997
    The value of acetylsalicylic acid (ASS) in the secondary prevention of ischemic stroke is well established. However, the optimum dose of AAS for stroke-threatened patients remains unsettled...
  55. ncbi The chemotherapy of rodent malaria. LXI. Drug combinations to impede the selection of drug resistance, part 4: the potential role of 8-aminoquinolines
    W Peters
    Centre for Tropical Antiprotozoal Chemotherapy, Y Block, Northwick Park Institute for Medical Research, Watford Road, Harrow HA1 3UJ, UK
    Ann Trop Med Parasitol 97:221-36. 2003
    ..chabaudi ASS (the 238C line) or P. yoelii ssp...
  56. ncbi Illustrated identification keys to strongylid parasites (Strongylidae: Nematoda) of horses, zebras and asses (Equidae)
    J Ralph Lichtenfels
    Animal Parasitic Diseases Laboratory, Agricultural Research Service, US Department of Agriculture, The Henry A Wallace Beltsville Agricultural Research Center, Beltsville, MD 20705 2350, USA
    Vet Parasitol 156:4-161. 2008
    The Equidae (the horse, Equus caballus, the ass, Equus asinus, zebras and their hybrids) are hosts to a great variety of nematode parasites, some of which can cause significant morbidity or mortality if individual hosts are untreated...
  57. pmc Genetic diversity in Spanish donkey breeds using microsatellite DNA markers
    J Aranguren-Mendez
    Unitat de Genètica i Millora Animal, Departament de Ciencia Animal i dels Aliments, Facultat de Veterinaria, Universitat Autonoma de Barcelona, 08193 Bellaterra, Barcelona, Spain
    Genet Sel Evol 33:433-42. 2001
    ..showed little differentiation between Spanish breeds, but great differentiation between them and the Moroccan ass and also with the horse, used as an outgroup...
  58. ncbi [Is sodium salicylate suitable as a protective agent in the analgesic-induced asthma syndrome?]
    A Schäufele
    Hochgebirgsklinik Davos Wolfgang, Schweiz
    Pneumologie 44:371-2. 1990
    ..In none of the patients was the asthmatic reaction to acetylsalicylic acid (ASS) completely inhibited by the oral administration of NaS...
  59. ncbi [Genetic analysis of ASS1, ASL and SLC25A13 in citrullinemia patients]
    Pengqiang Wen
    Shenzhen Institute of Pediatrics, Department of Neonatology, Department of Neurology, Department of Traditional Chinese Medicine, Shenzhen Children s Hospital, Shenzhen, Guangdong 518026, P R China email
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 31:268-71. 2014
    ..To detect potential mutations of Y9ASS1, ASL and SLC25A13 genes in four patients manifesting citrullinemia...
  60. pmc Argininosuccinate synthetase 1 depletion produces a metabolic state conducive to herpes simplex virus 1 infection
    Sarah L Grady
    Departments of Molecular Biology and Chemistry, and Lewis Sigler Institute for Integrative Genomics, Princeton University, Princeton, NJ 08544
    Proc Natl Acad Sci U S A 110:E5006-15. 2013
    ..The screen identified multiple enzymes predicted to influence HSV-1 replication, including argininosuccinate synthetase 1 (AS1), which consumes aspartate as part of de novo arginine synthesis...
  61. ncbi Application of mutation analysis for the previously uncertain cases of adult-onset type II citrullinemia (CTLN2) and their clinical profiles
    Toju Tanaka
    Department of Pediatrics, National Nishi Sapporo Hospital, Sapporo 063 0005, Japan
    Tohoku J Exp Med 198:89-97. 2002
    Type II citrullinemia (CTLN2) is characterized by a deficiency of argininosuccinate synthetase (ASS) in the liver. Mutation analysis of the SLC25A13 gene, which is responsible for CTLN2, provides a rapid and accurate diagnosis...
  62. doi Argininosuccinate synthase as a novel biomarker for inflammatory conditions
    Mengde Cao
    Banyan Laboratories, Inc, Alachua, FL 32615, USA
    Biomarkers 18:242-9. 2013
    b>Argininosuccinate synthase (ASS) plays an important role in regulating metabolic functions in mammals...
  63. ncbi Simultaneous determination of PPCPs, EDCs, and artificial sweeteners in environmental water samples using a single-step SPE coupled with HPLC-MS/MS and isotope dilution
    Ngoc Han Tran
    Department of Civil and Environmental Engineering, Faculty of Engineering, National University of Singapore, 1 Engineering Drive 2, E1A 02 19, Singapore 117576, Singapore
    Talanta 113:82-92. 2013
    ..and personal care products (PPCPs), endocrine disrupting chemicals (EDCs) and artificial sweeteners (ASs) was developed...
  64. ncbi Expression of three mitochondrial solute carriers, citrin, aralar1 and ornithine transporter, in relation to urea cycle in mice
    Laila Begum
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, 8 35 1 Sakuragaoka, 890 8520, Kagoshima, Japan
    Biochim Biophys Acta 1574:283-92. 2002
    ..in relation to urea cycle enzyme genes, carbamoylphosphate synthetase I (CPS) and argininosuccinate synthetase (ASS)...
  65. doi Occurrence of seven artificial sweeteners in the aquatic environment and precipitation of Tianjin, China
    Zhiwei Gan
    MOE Key Laboratory of Pollution Processes and Environmental Criteria, College of Environmental Science and Engineering, Nankai University, 94 Weijin Street, Tianjin 300071, China
    Water Res 47:4928-37. 2013
    ..and precipitation samples, from Tianjin, China, were analyzed for seven commonly used artificial sweeteners (ASs)...
  66. ncbi Functional-eicosanoid-test (FET) and disease
    H W Baenkler
    University Hospital, Department of Medicine 3 Immunology, Erlangen, Germany
    J Physiol Pharmacol 57:65-72. 2006
    ..Healthy controls rendered normal data. Patients with intolerance to acetylsalicylic acid (ASS) showed an elevated output of PG and LT upon stimulation. Addition of ASS shifted from PG to LT...
  67. pmc Arginine deiminase resistance in melanoma cells is associated with metabolic reprogramming, glucose dependence, and glutamine addiction
    Yan Long
    Corresponding Author Macus Tien Kuo, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Box 951, Houston, TX 77030
    Mol Cancer Ther 12:2581-90. 2013
    ..including melanomas, are auxotrophic for arginine due to reduced expression of argininosuccinate synthetase-1 (ASS1), the rate-limiting enzyme for arginine biosynthesis...
  68. ncbi Are rapid transitions between invasive and native species caused by alternative stable states, and does it matter?
    Gretchen J A Hansen
    Center for Limnology, University of Wisconsin, 680 North Park Street, Madison, Wisconsin 53706, USA
    Ecology 94:2207-19. 2013
    Rapid transitions in ecosystem structure, or regime shifts, are a hallmark of alternative stable states (ASS). However, regime shifts can occur even when feedbacks are not strong enough to cause ASS...
  69. ncbi Fundamental studies of electrochemically controlled surface oxidation and hydrophobicity of natural enargite
    Chris Plackowski
    School of Chemical Engineering, The University of Queensland, Brisbane, Queensland 4072, Australia
    Langmuir 29:2371-86. 2013
    The surface oxidation and hydrophobicity of natural enargite (Cu(3)AsS(4)) and the formation of oxidation species at the mineral surface have been examined by a novel experimental approach that combines electrochemical techniques and ..
  70. doi Creatine metabolism in urea cycle defects
    Sara Boenzi
    Division of Metabolism and Research Unit of Metabolic Biochemistry, Bambino Gesu Children s Hospital, IRCCS, Piazza S Onofrio 4, 00165, Rome, Italy
    J Inherit Metab Dis 35:647-53. 2012
    ..e., OTC, ASS, ASL deficiencies, HHH syndrome and lysinuric protein intolerance)...
  71. pmc Genetic analysis of audiogenic seizure susceptibility in C57BL/6J X DBA/2J recombinant inbred strains of mice
    T N Seyfried
    Genetics 94:701-18. 1980
    The inheritance of susceptibility to audiogenic seizures (ASs) was studied in the C57BL/6J (B6) and DBA/2J (D2) progenitor strains, their reciprocal F(1) hybrids, backcross generations and in 21 B6 x D2 recombinant inbred (RI) strains of ..
  72. pmc Establishment and comparison of combining disease and syndrome model of asthma with "kidney yang deficiency" and "abnormal savda"
    Bei Li
    Department of Integrative Medicine, Huashan Hospital, Fudan University, Shanghai 200040, China
    Evid Based Complement Alternat Med 2013:658364. 2013
    ..Kidney Yang Deficiency syndrome (KYDS) in traditional Chinese medicine (TCM) and abnormal savda syndrome (ASS) in traditional Uighur medicine (TUM)...
  73. ncbi [Secondary and primary prevention of coronary heart disease: platelet aggregation inhibitors and anticoagulants]
    W Kubler
    Abteilung Innere Medizin III Medizinische Universitätsklinik Bergheimer Str 58 69151 Heidelberg, Germany
    Z Kardiol 91:40-8. 2002
    ..According to the results of the trials and due to its low price aspirin (ASS) can be still considered as the drug of choice...
  74. ncbi [The beginnings and development of diagnostic imaging in nuclear medicine]
    L Stefanovic
    Institut za onkologiju Zavod za nuklearnu medicinu, 21204 Sremska Kamenica
    Med Pregl 54:289-96. 2001
    ..RECTILINEAR SCANNER: It was constructed by B. Cassen and ass. in 1949, when first scintiscans were obtained. Technical improvements have been made 1950-1956 (B. Cassen and ass...
  75. doi The availability and mobility of arsenic and antimony in an acid sulfate soil pasture system
    Matthew Tighe
    Agronomy and Soil Science, School of Environmental and Rural Science, University of New England, Armidale NSW 2351, Australia Electronic address
    Sci Total Environ 463:151-60. 2013
    ..The floodplain also contains areas of active and potential acid sulfate soils (ASS)...
  76. pmc The miR-17-92 cluster and its target THBS1 are differentially expressed in angiosarcomas dependent on MYC amplification
    Antoine Italiano
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA
    Genes Chromosomes Cancer 51:569-78. 2012
    Angiosarcomas (ASs) represent a heterogeneous group of malignant vascular tumors that may occur spontaneously as primary tumors or secondarily after radiation therapy or in the context of chronic lymphedema...
  77. ncbi Na2EuAs2S5, NaEuAsS4, and Na4Eu(AsS4)2: controlling the valency of arsenic in polysulfide fluxes
    Tarun K Bera
    Department of Chemistry, Northwestern University, Evanston, Illinois 60208, United States
    Inorg Chem 51:4293-9. 2012
    ..Na(2)EuAs(2)S(5) has [As(2)S(5)](4-) units, built of corner sharing AsS(3) pyramids, which are coordinated to Eu(2+) ions to give a two-dimensional (2D) layered structure...
  78. doi Sustained delivery and efficacy of polymeric nanoparticles containing osteopontin and bone sialoprotein antisenses in rats with breast cancer bone metastasis
    Victoria Elazar
    Department of Pharmaceutics, School of Pharmacy, Faculty of Medicine, The Hebrew University of Jerusalem, Jerusalem, Israel
    Int J Cancer 126:1749-60. 2010
    ..The ASs designed against OPN and BSP-II were successfully encapsulated in NP composed of the biodegradable and ..
  79. doi Epigenetic silencing of argininosuccinate synthetase confers resistance to platinum-induced cell death but collateral sensitivity to arginine auxotrophy in ovarian cancer
    Linda J Nicholson
    King s College London School of Medicine, Cancer Studies Division, The Rayne Institute, St Thomas Hospital, London, United Kingdom
    Int J Cancer 125:1454-63. 2009
    ..Down-regulation of expression of argininosuccinate synthetase (ASS1), the rate-limiting enzyme in the biosynthesis of arginine, has been associated with the development of platinum ..
  80. doi Differential gene expression in young and senescent endothelial cells under static and laminar shear stress conditions
    Gyeong In Mun
    Department of Molecular Medicine and Cell and Matrix Research Institute, BK21 Medical Education Program for Human Resources, Republic of Korea
    Free Radic Biol Med 47:291-9. 2009
    ..These genes included argininosuccinate synthetase 1, which was determined to be critical for both basal and LSS-induced NO production in young HUVECs...
  81. doi Additional-singleton interference in efficient visual search: a common salience route for detection and compound tasks
    Michael Zehetleitner
    Department of General Psychology, Ludwig Maximilians Universitat Munchen, Leopoldstr 13, 80802 Munich, Germany
    Atten Percept Psychophys 71:1760-70. 2009
    In efficient search for feature singleton targets, additional singletons (ASs) defined in a nontarget dimension are frequently found to interfere with performance...
  82. pmc Neural stem cells improve cognition via BDNF in a transgenic model of Alzheimer disease
    Mathew Blurton-Jones
    Department of Neurobiology and Behavior and Institute for Memory Impairments and Neurological Disorders, University of California, Irvine, CA 92697, USA
    Proc Natl Acad Sci U S A 106:13594-9. 2009
    ..Interestingly, despite widespread and established Ass plaque and neurofibrillary tangle pathology, hippocampal neural stem cell transplantation rescues the spatial ..
  83. doi Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients
    Andrea Váncsa
    Division of Rheumatology, 3rd Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Moricz Zs Krt 22, 4032, Debrecen, Hungary
    Rheumatol Int 29:989-94. 2009
    ..radiological findings and therapeutic response between the anti-SS-A positive and negative antisynthetase syndrome (ASS) patients. A prospective study of 315 IIM patients was conducted including 27 anti-Jo-1 positive ASS patients...
  84. pmc Conformational transitions of duplex and triplex nucleic acid helices: thermodynamic analysis of effects of salt concentration on stability using preferential interaction coefficients
    J P Bond
    Department of Chemistry, University of Wisconsin Madison 53706
    Biophys J 67:825-36. 1994
    ..aMS for the multi-stranded (ordered) conformations, we determined the corresponding best fitted values of bSS and aSS for single-stranded RNA and DNA. The resulting best fitted values of aSS are systematically less than aDS by 2-4 A...
  85. ncbi The study of sodium channels involved in pain responses using specific modulators
    Yong Hua Ji
    School of Life Sciences, Shanghai University, Shanghai 200444, China
    Sheng Li Xue Bao 60:628-34. 2008
    ..However, BmK IT2 and BmK ASs, potently suppressed both the peak TTX-resistant and TTX-sensitive Na(+) currents on rat small DRG neurons...
  86. pmc Sequence for human argininosuccinate synthetase cDNA
    H G Bock
    Nucleic Acids Res 11:6505-12. 1983
    ....
  87. ncbi Suppressed expression of the urea cycle enzyme genes in the liver of carnitine-deficient juvenile visceral steatosis (JVS) mice in infancy and during starvation in adulthood
    M Tomomura
    Department of Biochemistry, Faculty of Medicine, Kagoshima University
    J Biochem 121:172-7. 1997
    ..The steady-state levels of CPS and argininosuccinate synthetase (ASS) mRNAs in the liver of JVS mice were normalized by no later than 8 weeks...
  88. ncbi A two-dimensional gel database of human colon carcinoma proteins
    H Ji
    Joint Protein Structure Laboratory, Ludwig Institute for Cancer Research Melbourne Branch, Parkville, Victoria, Australia
    Electrophoresis 18:605-13. 1997
    ..These data, and descriptions of the micro-techniques employed in this laboratory for identifying 2-DE resolved proteins can be accessed via the internet URL: http:(/)/www.ludwig.edu.au...
  89. ncbi Neogenesis vs. apoptosis As main components of pancreatic beta cell ass changes in glucose-infused normal and mildly diabetic adult rats
    C Bernard
    Laboratoire de Physiopathologie de la Nutrition, CNRS ESA 7059, Universite Paris 7, Paris France
    FASEB J 13:1195-205. 1999
    ..They also show that changes in beta cell mass in ND and STZ rats resulted from a disruption in the balance between neogenesis and apoptosis...
  90. ncbi Expression of the genes of arginine-synthesizing enzymes in the rat kidney during development
    I Goutal
    Groupe de Biochimie et Physiologie Digestive et Nutritionnelle GBPDN, Institut Fédératif de Recherches Multidisciplinaires sur les Peptides no 23 IFRMP, UFR Médecine Pharmacie de Rouen, France
    Biol Neonate 76:253-60. 1999
    The expression of two genes, coding for argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL), enzymes which synthesize arginine, was studied by Northern analysis in various tissues of fetal rats...
  91. doi Potential of supramolecular solvents for the extraction of contaminants in liquid foods
    Ana Ballesteros-Gómez
    Department of Analytical Chemistry, Facultad de Ciencias, Edificio Anexo Marie Curie, Campus de Rabanales, 14071 Cordoba, Spain
    J Chromatogr A 1216:530-9. 2009
    ..The ASS selected was made up of reversed micelles of decanoic dispersed in tetrahydrofuran (THF)-water and the contaminants ..
  92. ncbi Molecular basis of enzyme abnormalities in urea cycle disorders. With special reference to citrullinemia and argininosuccinic aciduria
    T Saheki
    Department of Biochemistry, Faculty of Medicine, Kagoshima University
    Enzyme 38:227-32. 1987
    ..Metab. Dis. 8: 155-156, 1985] into three types from the properties of the deficient argininosuccinate synthetase (ASS) of the patients...
  93. ncbi Association constants and distribution functions for ion pairs in binary solvent mixtures: application to a cyanine dye system
    A V Odinokov
    Photochemistry Center, RAS, ul Novatorov 7a, 119421 Moscow, Russia
    J Chem Phys 135:144503. 2011
    The computations of the association constants K(ass) were performed at the microscopic level for the ion pair Cy(+)I(-) composed of the complex cyanine dye cation Cy(+) coupled to the negative iodine counterion...
  94. doi Arginine or citrulline associated with a statin stimulates nitric oxide production in bovine aortic endothelial cells
    Marie Clotilde Berthe
    Laboratoire de Biologie de la Nutrition, EA2498, Universite Paris Descartes, France
    Eur J Pharmacol 670:566-70. 2011
    ..is an antiatherogenic vasodilator synthesized from arginine and, indirectly, from citrulline through argininosuccinate synthase (ASS) and argininosuccinate lyase (ASL)...
  95. doi Human SCCA serpins inhibit staphylococcal cysteine proteases by forming classic "serpin-like" covalent complexes
    Tomasz Kantyka
    Department of Microbiology, Faculty of Biochemistry, Biophysics, and Biotechnology, Jagiellonian University, Krakow, Poland
    Methods Enzymol 499:331-45. 2011
    ..The high association rate constant (k(ass)) for inhibitory complex formation (1.9 × 10⁴ and 5...
  96. pmc Alternative stable states generated by ontogenetic niche shift in the presence of multiple resource use
    Takefumi Nakazawa
    Center for Ecological Research, Kyoto University, Otsu, Japan
    PLoS ONE 6:e14667. 2011
    It has been suggested that when juveniles and adults use different resources or habitats, alternative stable states (ASS) may exist in systems coupled by an ontogenetic niche shift. However, mainly the simplest system, i.e...
  97. doi Citrullinaemia type I: a common mutation in the Pacific Island population
    Emma Glamuzina
    Paediatric Metabolic Service, Starship Children s Hospital, Auckland, New Zealand
    J Paediatr Child Health 47:262-5. 2011
    The aim of this study was to develop and apply a mutation screening protocol for the ASS1 gene in order to confirm the diagnosis of citrullinaemia type I in neonates with elevated citrulline on expanded newborn screening (E-NBS).
  98. ncbi Arginine deiminase enhances MCF-7 cell radiosensitivity by inducing changes in the expression of cell cycle-related proteins
    Hwan Park
    Department of Pharmacology and BK21 Program for Medical Sciences, College of Medicine, Korea University, Seoul 136 705, Korea
    Mol Cells 25:305-11. 2008
    ..did not suppress the growth of MCF-7 cells due to the high level of expression of argininosuccinate synthetase (ASS), which convert citrulline, a product of arginine degradation by ADI, to arginine...
  99. doi A pericentric inv(9)(p22q34) of the der(9)t(9;22)(q34;q11.2) is a recurrent secondary anomaly in Ph-positive leukemia
    Jinlan Pan
    The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis, 188 Shizi Street, Suzhou 215006, PR China
    Cancer Genet Cytogenet 203:333-40. 2010
    ..5% of bone marrow cells in the AML patient, but did not detect the deletion of the sequence of ASS/9q34 in these four patients...
  100. pmc Two hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes
    Carlos J Perez
    Department of Carcinogenesis, The University of Texas MD Anderson Cancer Center, Smithville, Texas 78957, USA
    Am J Pathol 177:1958-68. 2010
    Citrullinemia type I (CTLN1, OMIM# 215700) is an inherited urea cycle disorder that is caused by an argininosuccinate synthetase (ASS) enzyme deficiency...

Research Grants78

  1. Arginine Deprivation: A targeted therapy for Melanoma
    Lynn Feun; Fiscal Year: 2006
    ..is primarily due to the fact that melanoma cells, unlike normal cells, do not express argininosuccinate synthetase (ASS) and hence are unable to synthesize arginine...
  2. Biomarker for liver ischemia/reperfusion-induced injury
    MONIKA OLI; Fiscal Year: 2006
    ..The most promising biomarker candidate discovered to date is an enzyme of urea/nitric oxide cycle argininosuccinate synthase (ASS)...
  3. Pathologic Interactions of Amyloid Beta-Protein
    William E Van Nostrand; Fiscal Year: 2010
    Abnormal accumulation and deposition of the amyloid ss-protein (Ass), a prominent pathological feature of patients with Alzheimer's disease (AD) and related disorders, can occur from increased production but in most cases is likely ..
  4. ASS, NOS2, AND ALCOHOLIC LIVER DISEASE
    Natalia Nieto; Fiscal Year: 2010
    ..We have identified argininosuccinate synthase (ASS) as up-regulated by chronic alcohol feeding...
  5. Impact of Antibiotics and Vaccines on the in vivo Evolution of S. pneumoniae
    Garth Ehrlich; Fiscal Year: 2009
    ..n2 500 -o o_- OIL iris OCD ton N-4 :,m 0>E ass= a foil QC) yob Computat'++ional Personnel ComEputational Personnel p0) the NCBI as which we use for our ..
  6. ASS, NOS2, AND ALCOHOLIC LIVER DISEASE
    Natalia Nieto; Fiscal Year: 2009
    ..We have identified argininosuccinate synthase (ASS) as up-regulated by chronic alcohol feeding...
  7. ASS, NOS2, AND ALCOHOLIC LIVER DISEASE
    Natalia Nieto; Fiscal Year: 2009
    ..We have identified argininosuccinate synthase (ASS) as up-regulated by chronic alcohol feeding...
  8. ASS, NOS2, AND ALCOHOLIC LIVER DISEASE
    Natalia Nieto; Fiscal Year: 2012
    ..We have identified argininosuccinate synthase (ASS) as up-regulated by chronic alcohol feeding...
  9. Arginine Deprivation: A targeted therapy for Melanoma
    Lynn Feun; Fiscal Year: 2004
    ..is primarily due to the fact that melanoma cells, unlike normal cells, do not express argininosuccinate synthetase (ASS) and hence are unable to synthesize arginine...
  10. Arginine Deprivation: A targeted therapy for Melanoma
    Lynn Feun; Fiscal Year: 2005
    ..is primarily due to the fact that melanoma cells, unlike normal cells, do not express argininosuccinate synthetase (ASS) and hence are unable to synthesize arginine...
  11. HEPATOCYTE GENE THERAPY FOR CITRULLINEMIA
    Brendan Lee; Fiscal Year: 2001
    ..Citrullinemia, an autosomal recessive enzymatic deficiency of argininosuccinate synthetase (ASS), is representative of the group of urea cycle defects...
  12. HEPATOCYTE GENE THERAPY FOR CITRULLINEMIA
    Brendan Lee; Fiscal Year: 2000
    ..Citrullinemia, an autosomal recessive enzymatic deficiency of argininosuccinate synthetase (ASS), is representative of the group of urea cycle defects...
  13. HEPATOCYTE GENE THERAPY FOR CITRULLINEMIA
    Brendan Lee; Fiscal Year: 1999
    ..Citrullinemia, an autosomal recessive enzymatic deficiency of argininosuccinate synthetase (ASS), is representative of the group of urea cycle defects...
  14. HEPATOCYTE GENE THERAPY FOR CITRULLINEMIA
    Brendan Lee; Fiscal Year: 2000
    ..Citrullinemia, an autosomal recessive enzymatic deficiency of argininosuccinate synthetase (ASS), is representative of the group of urea cycle defects...
  15. HEPATOCYTE GENE THERAPY FOR CITRULLINEMIA
    Brendan Lee; Fiscal Year: 1999
    ..Citrullinemia, an autosomal recessive enzymatic deficiency of argininosuccinate synthetase (ASS), is representative of the group of urea cycle defects...
  16. Novel diagnostic and safety biomarkers of liver injury and hepatotoxicity
    Stanislav I Svetlov; Fiscal Year: 2010
    ..We identified several novel liver-specific biomarkers in rat models of hepatic injury with the argininosuccinate synthase (ASS) and hepatic estrogen sulfotransferases (EST-1) being the most sensitive markers which rapidly ..
  17. Novel diagnostic and safety biomarkers of liver injury and hepatotoxicity
    STANISLAV SVETLOV; Fiscal Year: 2009
    ..We identified several novel liver-specific biomarkers in rat models of hepatic injury with the argininosuccinate synthase (ASS) and hepatic estrogen sulfotransferases (EST-1) being the most sensitive markers which rapidly ..
  18. Novel diagnostic and safety biomarkers of liver injury and hepatotoxicity
    STANISLAV SVETLOV; Fiscal Year: 2009
    ..We identified several novel liver-specific biomarkers in rat models of hepatic injury with the argininosuccinate synthase (ASS) and hepatic estrogen sulfotransferases (EST-1) being the most sensitive markers which rapidly ..
  19. Nitric Oxide Precursors and Congenital Heart Surgery
    Frederick Barr; Fiscal Year: 2004
    ..enzymes carbamyl phosphate synthetase I (CPSl), ornithine transcarbamylase (OTC), argininosuccinate syntheatse (ASS), argininosuccinate lyase (ASL), and enodothelial nitric oxide synthase (eNOS) influence arginine and NO ..
  20. Nitric Oxide Precursors and Congenital Heart Surgery
    Frederick Barr; Fiscal Year: 2005
    ..enzymes carbamyl phosphate synthetase I (CPSl), ornithine transcarbamylase (OTC), argininosuccinate syntheatse (ASS), argininosuccinate lyase (ASL), and enodothelial nitric oxide synthase (eNOS) influence arginine and NO ..
  21. Nitric Oxide Precursors and Congenital Heart Surgery
    Frederick Barr; Fiscal Year: 2006
    ..enzymes carbamyl phosphate synthetase I (CPSl), ornithine transcarbamylase (OTC), argininosuccinate syntheatse (ASS), argininosuccinate lyase (ASL), and enodothelial nitric oxide synthase (eNOS) influence arginine and NO ..
  22. Nitric Oxide Precursors and Congenital Heart Surgery
    Frederick Barr; Fiscal Year: 2007
    ..enzymes carbamyl phosphate synthetase I (CPSl), ornithine transcarbamylase (OTC), argininosuccinate syntheatse (ASS), argininosuccinate lyase (ASL), and enodothelial nitric oxide synthase (eNOS) influence arginine and NO ..
  23. History of Alzheimer predisposes children to amyloid beta-related hypometabolism
    Lisa Mosconi; Fiscal Year: 2013
    ..Our first aim will be to examine whether NL FHm show reduced CMRglc, increased Ass deposition and increased oxidative stress vs FH- and FHp, and whether the changes in these variables are related...
  24. Cyclohexanehexol Therapy in Transgenic Models of Alzheimer's Disease
    Alpaslan Dedeoglu; Fiscal Year: 2013
    Project Summary/Abstract Senile plaques that contain beta amyloid (Ass) and neurofibrillary tangles (NFT) with phosphorylated tau are the pathological hallmarks of Alzheimer's disease (AD)...
  25. Fyn Inhibition by AZD0530 for Alzheimers Disease
    Christopher H van Dyck; Fiscal Year: 2013
    ..Multiple studies implicate Fyn kinase in the synaptic pathophysiology of AD, with links to both Ass and Tau pathology...
  26. Small Molecule Development of PrPc Antagonists for the Treatment of Alzheimer's D
    Stephen M Strittmatter; Fiscal Year: 2013
    ..The hypothesis that the Amyloid beta (Ass) peptide plays an early causative role is supported by pathology, by human genetics and by biomarker studies...
  27. AGING IN THE BRAIN: ROLE OF THE FIBROUS PROTEINS
    Dennis J Selkoe; Fiscal Year: 2013
    ..APP mutations or duplications cause rare but mechanistically important forms of AD, and its Ass product accumulates in all AD patients...
  28. Role of lipid intermediates in the limited human adipose tissue expandability ass
    Angela R Subauste; Fiscal Year: 2013
    ..At the completion of these studies we will have defined the faulty lipid signal present in obese insulin-resistant individuals. ..
  29. N-terminus of sAPP Regulates Abeta Assembly
    William E Van Nostrand; Fiscal Year: 2013
    Abnormal accumulation, assembly and deposition of the amyloid ss-protein (Ass) is a prominent pathological feature of patients with Alzheimer's disease (AD) and related disorders...
  30. Mouse Model of Myelin Basic Protein-Amyloid Beta Interactions in Brain
    William E Van Nostrand; Fiscal Year: 2013
    Accumulation of the amyloid ss-protein (Ass) in brain, either as parenchymal plaques or cerebrovascular deposits, is a key pathological feature of patients with Alzheimer's disease (AD) and several related disorders...
  31. Characterizing the Evolution of Amyloid Deposition in Normal Elderly
    Keith A Johnson; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): The deposition of amyloid-beta (Ass) deposition is thought to begin many years prior to the onset of clinical Alzheimer's disease, but until recently it was not possible to track the ..
  32. Cyclohexanehexol Therapy in Transgenic Models of Alzheimer's Disease
    Alpaslan Dedeoglu; Fiscal Year: 2010
    ..b>Ass peptides, especially A42, are thought to play a key role in the pathogenesis of AD...
  33. Cyclohexanehexol Therapy in Transgenic Models of Alzheimer's Disease
    Alpaslan Dedeoglu; Fiscal Year: 2009
    ..b>Ass peptides, especially A42, are thought to play a key role in the pathogenesis of AD...
  34. CELL-SURFACE PROTEINS IN CAMPYLOBACTER FETUS VIRULENCE
    Geraldine Miller; Fiscal Year: 1990
    Human campylobacter infections have been increasingly recognized ass major causes of human illness. Campylobacter jejuni is now recognized as the most common bacterial cause of invasive diarrhea in the United States...
  35. AFFERENT MECHANISMS UNDERLYING BLADDER PAIN
    WILLIAM DEGROAT; Fiscal Year: 2007
    ..Associate Alyssa Hawranko Naoki Yoshimura " " Res. Ass. Prof. Kyu-Sung Lee " " Res. Associate Yao-Chi Chuang " " Visiting Ass. Prof. Jon Beckel " " Res...
  36. AFFERENT MECHANISMS UNDERLYING BLADDER PAIN
    WILLIAM DEGROAT; Fiscal Year: 2009
    ..Associate Alyssa Hawranko Naoki Yoshimura " " Res. Ass. Prof. Kyu-Sung Lee " " Res. Associate Yao-Chi Chuang " " Visiting Ass. Prof. Jon Beckel " " Res...
  37. DYSTONIA AND TUBEROUS SCLEROSIS--LOCALIZATION ON 9Q32-34
    ELIZABETH HENSKE; Fiscal Year: 1991
    ..linkage for early-onset Jewish and non-Jewish dystonia has been observed to the arginino-succinate synthetase (ASS) locus on 9q34. The disease gene is believed to lie In the 5 cM interval between the AK-1 and ASS loci...
  38. A first-in-class orally active anti-TNF-alpha inhibitor to treat AD
    Marni E Harris-White; Fiscal Year: 2013
    ..Determine the effect of PD2015 on indicators of neuroinflammation and AD- associated pathology including TNF-[unreadable] levels, Ass1-40/Ass1-42 levels, microglial activation, tau, phospho-tau, synaptophysin, SNAP-25 in 3xTg AD mice.
  39. WGA as a Novel Vehicle for Intranasal Delivery of an Anti-A-beta Antibody in AD
    Neelima B Chauhan; Fiscal Year: 2012
    ..Passive immunization by intravenous injection of Ass monoclonal antibodies (mAb) has shown promising results such as lowering of cerebral Ass and stabilization or ..
  40. Early Detection of Cerebral Amyloid Angiopathy
    Steven M Greenberg; Fiscal Year: 2010
    Deposition of ss-amyloid (Ass) in the cerebral vessels (cerebral amyloid angiopathy or CAA) is a major cause of hemorrhagic stroke, a contributor to vascular cognitive impairment, and a complicating factor in attempts to develop anti-..
  41. Pathological Influence of Vasculotropic Mutant Amyloid-Beta
    William E Van Nostrand; Fiscal Year: 2011
    ..peptides in brain and develop abundant amyloid pathology or 2) low, physiological levels of human wild-type Ass peptides in brain with no amyloid pathology...
  42. Pathological Influence of Vasculotropic Mutant Amyloid-Beta
    William E Van Nostrand; Fiscal Year: 2010
    ..A peptides in brain and develop abundant amyloid pathology or 2) low, physiological levels of human wild-type Ass peptides in brain with no amyloid pathology...
  43. Pathological Influence of Vasculotropic Mutant Amyloid-Beta
    William Van Nostrand; Fiscal Year: 2009
    ..A peptides in brain and develop abundant amyloid pathology or 2) low, physiological levels of human wild-type Ass peptides in brain with no amyloid pathology...
  44. IMPAIRED TUMOR IMMUNITY DURING H PYLORI INFECTION
    VICTOR REYES; Fiscal Year: 2001
    ..class II MHC expression increased in parallel with the rise of local CD4+ T cell numbers and epithelial c;ass II MHC expression during infection with H. pylori...
  45. IMPAIRED TUMOR IMMUNITY DURING H PYLORI INFECTION
    VICTOR REYES; Fiscal Year: 2000
    ..class II MHC expression increased in parallel with the rise of local CD4+ T cell numbers and epithelial c;ass II MHC expression during infection with H. pylori...
  46. IMPAIRED TUMOR IMMUNITY DURING H PYLORI INFECTION
    VICTOR REYES; Fiscal Year: 2002
    ..class II MHC expression increased in parallel with the rise of local CD4+ T cell numbers and epithelial c;ass II MHC expression during infection with H. pylori...
  47. IMPAIRED TUMOR IMMUNITY DURING H PYLORI INFECTION
    VICTOR REYES; Fiscal Year: 2003
    ..class II MHC expression increased in parallel with the rise of local CD4+ T cell numbers and epithelial c;ass II MHC expression during infection with H. pylori...
  48. IMPAIRED TUMOR IMMUNITY DURING H PYLORI INFECTION
    VICTOR REYES; Fiscal Year: 1999
    ..class II MHC expression increased in parallel with the rise of local CD4+ T cell numbers and epithelial c;ass II MHC expression during infection with H. pylori...
  49. RECOVERY OF FUNCTION AND STRUCTURE AFTER ACOUSTIC TRAUMA
    Richard Salvi; Fiscal Year: 1992
    ..All information reaching the higher levels of the auditory system must ass through the cochlear ganglion, and cells in the cochlear ganglion are most intimately related to the cochlear hair ..
  50. New Strategy to Fight Selective Cholinergic Neuronal Loss in Alzheimer Disease
    William Z Suo; Fiscal Year: 2013
    ..GRK5 deficiency also exaggerates Ass accumulation and gliosis...
  51. Molecular Basis of mu-Opioid Receptor Gene Regulation
    Jane Ko; Fiscal Year: 2004
    ..Several cis-elements have been identified, including the ds and ass cis-elements, in the proximal promoter region...
  52. Molecular Basis of mu-Opioid Receptor Gene Regulation
    Jane Ko; Fiscal Year: 2006
    ..Several cis-elements have been identified, including the ds and ass cis-elements, in the proximal promoter region...
  53. Molecular Basis of mu-Opioid Receptor Gene Regulation
    Jane Ko; Fiscal Year: 2005
    ..Several cis-elements have been identified, including the ds and ass cis-elements, in the proximal promoter region...
  54. Molecular Basis of mu-Opioid Receptor Gene Regulation
    Jane Ko; Fiscal Year: 2007
    ..Several cis-elements have been identified, including the ds and ass cis-elements, in the proximal promoter region...
  55. Molecular Basis of mu-Opioid Receptor Gene Regulation
    Jane Ko; Fiscal Year: 2009
    ..Several cis-elements have been identified, including the ds and ass cis-elements, in the proximal promoter region...
  56. Development of protease inhibitor drugs to treat Alzheimer's disease
    Gregory Hook; Fiscal Year: 2009
    ..Neurotoxic ss-amyloid peptides (Ass) are thought to cause the disease with their accumulation in brain plaques being a hallmark...
  57. VISUAL PROCESSING & SMOOTH EYE MOVEMENTS
    MICHAEL MUSTARI; Fiscal Year: 1999
    ..eye movements syndromes such as latent nystagmus, smooth pursuit asymmetry, strabismus and gaze instability, often ass ociated with problems in the early development of binocular vision...
  58. Novel Mouse Model to Study Myelin Basic Protein-Amyloid Beta Protein Interactions
    William E Van Nostrand; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Accumulation of the amyloid ss-protein (Ass) in brain, either as parenchymal plaques or cerebrovascular deposits, is a key pathological feature of patients with Alzheimer's disease (AD) and ..
  59. Maternal history of Alzheimer's predisposes children to amyloid beta-related hy
    Lisa Mosconi; Fiscal Year: 2010
    ..Our first aim will be to examine whether NL FHm show reduced CMRglc, increased Ass deposition and increased oxidative stress vs FH- and FHp, and whether the changes in these variables are related...
  60. Transmission-blocking vaccine for HIV-1
    Robert C Rose; Fiscal Year: 2010
    ..Since other amyloid fibrils (Ass) have adjuvant-like immunostimulatory properties, we will also test whether these mutated fibrils elicit the ..
  61. Transmission-blocking vaccine for HIV-1
    Robert C Rose; Fiscal Year: 2010
    ..Since other amyloid fibrils (Ass) have adjuvant-like immunostimulatory properties, we will also test whether these mutated fibrils elicit the ..
  62. Transmission-blocking vaccine for HIV-1
    Stephen Dewhurst; Fiscal Year: 2009
    ..Since other amyloid fibrils (Ass) have adjuvant-like immunostimulatory properties, we will also test whether these mutated fibrils elicit the ..
  63. Structure and conformational diversity of amyloid oligomers
    Charles G Glabe; Fiscal Year: 2010
    ..of amyloid deposits accumulate in disease brain and current evidence suggests that soluble, oligomeric forms of Ass may play primary role in pathogenesis...
  64. Characterizing the Evolution of Amyloid Deposition in Normal Elderly
    Keith A Johnson; Fiscal Year: 2010
    The deposition of amyloid-beta (Ass) deposition is thought to begin many years prior to the onset of clinical Alzheimer's disease, but until recently it was not possible to track the longitudinal accumulation of Ass in vivo...
  65. Educational Tools and Facilitation of IRB Activitie
    JOHN OCONNOR; Fiscal Year: 2002
    ..increasing IRB efficiency by facilitating certain aspects of the review process, decreasing IRB workload by ass igning educational and training /functions to a newly formed Responsible Conduct of Research Committeect which will ..
  66. Uptake of STAR: Increasing Qualified Staff to improve care of older adults in Ass
    Linda Teri; Fiscal Year: 2009
    ..The proposed project is a partnership between university researchers and a community organization to translate STAR for broader, national use. ..
  67. Uptake of STAR: Increasing Qualified Staff to improve care of older adults in Ass
    Linda Teri; Fiscal Year: 2010
    ..The proposed project is a partnership between university researchers and a community organization to translate STAR for broader, national use. ..
  68. Pathologic Interactions of Amyloid Beta-Protein
    William E Van Nostrand; Fiscal Year: 2011
    Abnormal accumulation and deposition of the amyloid ss-protein (Ass), a prominent pathological feature of patients with Alzheimer's disease (AD) and related disorders, can occur from increased production but in most cases is likely ..
  69. Pathologic Interactions of Amyloid Beta-Protein
    William Van Nostrand; Fiscal Year: 2009
    Abnormal accumulation and deposition of the amyloid ss-protein (Ass), a prominent pathological feature of patients with Alzheimer's disease (AD) and related disorders, can occur from increased production but in most cases is likely ..
  70. NEUROTROPHIN GENE REGULATION IN THE INNER EAR
    Neil Segil; Fiscal Year: 1999
    ..However, difficulties associated with the introduction of foreign DNA (transfection) into post-mitotic cells such ass the hair cells, support cells, and the sensory neurons of the inner ear have so far precluded a detailed analysis ..
  71. GROWTH HORMONE REGULATION OF PUBERTY & FERTILITY
    Mark Wilson; Fiscal Year: 2000
    ..eye movements syndromes such as latent nystagmus, smooth pursuit asymmetry, strabismus and gaze instability, often ass ociated with problems in the early development of binocular vision...
  72. NEUROTROPHIN GENE REGULATION IN THE INNER EAR
    Neil Segil; Fiscal Year: 2000
    ..However, difficulties associated with the introduction of foreign DNA (transfection) into post-mitotic cells such ass the hair cells, support cells, and the sensory neurons of the inner ear have so far precluded a detailed analysis ..
  73. Inhibitors of Human Pal mitoyl Acyltransferase
    CHARLES DUCKER; Fiscal Year: 2005
    ..compounds that inhibit hPAT activity by screening a diverse collection of small molecules in an established ass ay and a high-throughput yeast-based assay. 2...
  74. TBI: Uncovering Mechanisms of AD-like pathology
    DOUGLAS HAMILTON SMITH; Fiscal Year: 2010
    ..risk of developing Alzheimer's disease (AD), and TBI can induce the rapid formation of amyloid-ss (Ass) plaques, a hallmark pathology of AD...