arginine and Inborn Urea Cycle Disorder

arginine has been researched along with Inborn Urea Cycle Disorder in 18 studies

Research

Studies (18)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (5.56)29.6817
2010's12 (66.67)24.3611
2020's5 (27.78)2.80

Authors

AuthorsStudies
Bechter, M; Cederbaum, SD; Diaz, GA1
Arnoux, JB; Benoist, JF; Bérat, CM; Bouchereau, J; Brassier, A; de Lonlay, P; Imbard, A; Josse, C; Montestruc, F; Pontoizeau, C; Schiff, M1
Alhasan, K; Bagga, A; Basu, RK; Bedoyan, JK; Bunchman, T; Chakraborty, R; Filler, G; Grewal, MK; Jouvet, P; Krishnappa, V; Lichter-Konecki, U; Machado, MC; McCulloch, M; Mew, NA; Picca, S; Raina, R; Schaefer, F; Sethi, SK; Vemuganti, M; Warady, BA1
Alvarez-Perez, JC; Avizonis, D; Badur, MG; Bird, GH; Bridon, G; Choi, DW; Danial, NN; Dhe-Paganon, S; Evans, L; Ficarro, SB; Fu, A; Garcia-Ocana, A; James Shapiro, AM; Jones, RG; Kamphorst, JJ; Karakose, E; Kin, T; Marto, JA; Rosselot, C; Seo, HS; Stewart, AF; Walensky, LD1
Caldovic, L; Haskins, N; McNutt, M; Morizono, H; Senkevitch, E; Sonaimuthu, P; Tuchman, M; Uapinyoying, P1
Cynober, L; Forbes, A; Osowska, S; Papadia, C1
Ono, H; Shigematsu, Y; Tamada, T1
Burgard, P; Chapman, KA; Gleich, F; Kölker, S; Lund, AM; Molema, F; Rizopoulos, D; Summar, ML; van der Ploeg, AT; Williams, M1
Ballhausen, D; Baumgartner, MR; Beblo, S; Burgard, P; Das, A; Gautschi, M; Glahn, EM; Grünert, SC; Häberle, J; Hennermann, J; Hochuli, M; Huemer, M; Karall, D; Kölker, S; Lachmann, RH; Lindner, M; Lotz-Havla, A; Möslinger, D; Nuoffer, JM; Plecko, B; Rüegger, CM; Rutsch, F; Santer, R; Spiekerkoetter, U; Staufner, C; Stricker, T; Wijburg, FA; Williams, M1
Blair, NF; Cremer, PD; Tchan, MC1
Calvello, R; Marobbio, CM; Palmieri, F; Palmieri, L; Panaro, MA; Pierri, CL; Punzi, G1
Dubrey, SW; Pal, S; Singh, S1
Brum, JM; Carvalho, DR; Coskun, T; Donner, MG; Gemperle, C; Häberle, J; Hersberger, M; Huemer, M; Karall, D; Riesner, B; Schlune, A; Scholl-Bürgi, S; Schrager, NL; Ulmer, H; Ünal, Ö; Weisfeld-Adams, JD1
Beblo, S; Burlina, A; Das, A; de Lonlay, P; Derbinski, J; Häberle, J; Hoffmann, GF; Konstantopoulou, V; Mayorandan, S; Rennecke, J; Thimm, E; Unsinn, C; Valayannopoulos, V1
Walker, V1
Erez, A; Lee, B; Nagamani, SC1
Dionisi-Vici, C; Ogier de Baulny, H; Schiff, M1
Boenzi, S; Boiani, A; Dionisi-Vici, C; Goffredo, BM; Martinelli, D; Pastore, A; Rizzo, C1

Reviews

6 review(s) available for arginine and Inborn Urea Cycle Disorder

ArticleYear
The role and control of arginine levels in arginase 1 deficiency.
    Journal of inherited metabolic disease, 2023, Volume: 46, Issue:1

    Topics: Arginase; Arginine; Child, Preschool; Humans; Hyperammonemia; Hyperargininemia; Urea Cycle Disorders, Inborn

2023
Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy.
    Nature reviews. Nephrology, 2020, Volume: 16, Issue:8

    Topics: Arginine; Carnitine; Child; Child, Preschool; Continuous Renal Replacement Therapy; Delphi Technique; Diet, Protein-Restricted; Humans; Hybrid Renal Replacement Therapy; Hyperammonemia; Infant; Infant, Newborn; Parenteral Nutrition; Peritoneal Dialysis; Phenylacetates; Phenylbutyrates; Practice Guidelines as Topic; Renal Dialysis; Sodium Benzoate; Urea Cycle Disorders, Inborn; Vitamin B Complex

2020
Citrulline in health and disease. Review on human studies.
    Clinical nutrition (Edinburgh, Scotland), 2018, Volume: 37, Issue:6 Pt A

    Topics: Anabolic Agents; Animals; Arginine; Athletic Performance; Carbamoyl-Phosphate Synthase I Deficiency Disease; Cardiovascular Diseases; Citrulline; Endothelial Cells; Enterocytes; Female; Humans; Immunity; Male; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Oxidative Stress; Protein Biosynthesis; Urea Cycle Disorders, Inborn

2018
Ammonia toxicity and its prevention in inherited defects of the urea cycle.
    Diabetes, obesity & metabolism, 2009, Volume: 11, Issue:9

    Topics: Adult; Ammonia; Animals; Arginine; Genetic Therapy; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Disease; Urea; Urea Cycle Disorders, Inborn

2009
Argininosuccinate lyase deficiency-argininosuccinic aciduria and beyond.
    American journal of medical genetics. Part C, Seminars in medical genetics, 2011, Feb-15, Volume: 157C, Issue:1

    Topics: Arginase; Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Carboxy-Lyases; Humans; Hyperammonemia; Infant, Newborn; Liver Diseases; Neonatal Screening; Nitric Oxide Synthase; Ornithine; Urea Cycle Disorders, Inborn

2011
Lysinuric protein intolerance (LPI): a multi organ disease by far more complex than a classic urea cycle disorder.
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:1

    Topics: Arginine; Humans; Immune System; Kidney Diseases; Large Neutral Amino Acid-Transporter 1; Lung; Lymphohistiocytosis, Hemophagocytic; Lysine; Macrophage Activation Syndrome; Urea Cycle Disorders, Inborn

2012

Other Studies

12 other study(ies) available for arginine and Inborn Urea Cycle Disorder

ArticleYear
Citrulline in the management of patients with urea cycle disorders.
    Orphanet journal of rare diseases, 2023, 07-21, Volume: 18, Issue:1

    Topics: Ammonia; Arginine; Citrulline; Drug-Related Side Effects and Adverse Reactions; Humans; Retrospective Studies; Urea; Urea Cycle Disorders, Inborn

2023
Glucose-dependent partitioning of arginine to the urea cycle protects β-cells from inflammation.
    Nature metabolism, 2020, Volume: 2, Issue:5

    Topics: Adolescent; Adult; Aged; Arginine; Aspartic Acid; Cell Survival; Citric Acid Cycle; Female; Glucose; Humans; Inflammation; Insulin-Secreting Cells; Male; Metabolomics; Middle Aged; Nitric Oxide; Pyruvate Carboxylase; Urea; Urea Cycle Disorders, Inborn; Young Adult

2020
Gene delivery corrects N-acetylglutamate synthase deficiency and enables insights in the physiological impact of L-arginine activation of N-acetylglutamate synthase.
    Scientific reports, 2021, 02-11, Volume: 11, Issue:1

    Topics: Amino-Acid N-Acetyltransferase; Animals; Arginine; Citrulline; Dependovirus; Disease Models, Animal; Gene Transfer Techniques; Glutamates; Humans; Hyperammonemia; Mice; Mice, Knockout; Mutant Proteins; Urea; Urea Cycle Disorders, Inborn

2021
Lactate/pyruvate in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
    Pediatrics international : official journal of the Japan Pediatric Society, 2018, Volume: 60, Issue:8

    Topics: Arginine; Diet, Protein-Restricted; Humans; Hyperammonemia; Infant; Lactates; Male; Ornithine; Pyruvates; Urea Cycle Disorders, Inborn

2018
Decreased plasma l-arginine levels in organic acidurias (MMA and PA) and decreased plasma branched-chain amino acid levels in urea cycle disorders as a potential cause of growth retardation: Options for treatment.
    Molecular genetics and metabolism, 2019, Volume: 126, Issue:4

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Arginine; Body Height; Child; Child, Preschool; Diet; Europe; Female; Growth Disorders; Humans; Longitudinal Studies; Male; Propionic Acidemia; Registries; Urea Cycle Disorders, Inborn

2019
Cross-sectional observational study of 208 patients with non-classical urea cycle disorders.
    Journal of inherited metabolic disease, 2014, Volume: 37, Issue:1

    Topics: Adolescent; Adult; Age of Onset; Aged; Arginine; Child; Child, Preschool; Citrulline; Cognition Disorders; Cohort Studies; Cross-Sectional Studies; Diet Therapy; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; Neonatal Screening; Ornithine Carbamoyltransferase Deficiency Disease; Time Factors; Treatment Outcome; Urea Cycle Disorders, Inborn; Young Adult

2014
Urea cycle disorders: a life-threatening yet treatable cause of metabolic encephalopathy in adults.
    Practical neurology, 2015, Volume: 15, Issue:1

    Topics: Adult; Ammonia; Arginine; Brain Diseases, Metabolic; Diet, Protein-Restricted; Fat Emulsions, Intravenous; Female; Glucose; Humans; Postpartum Period; Sodium Benzoate; Urea Cycle Disorders, Inborn

2015
Pathogenic potential of SLC25A15 mutations assessed by transport assays and complementation of Saccharomyces cerevisiae ORT1 null mutant.
    Molecular genetics and metabolism, 2015, Volume: 115, Issue:1

    Topics: Amino Acid Transport Systems, Basic; Arginine; Biological Transport; Culture Media; Escherichia coli; Genetic Complementation Test; Humans; Hyperammonemia; Liposomes; Mitochondrial Membrane Transport Proteins; Mutation, Missense; Ornithine; Recombinant Proteins; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Urea Cycle Disorders, Inborn

2015
Unusual cause of general malaise: a young woman with ornithine transcarbamylase deficiency.
    BMJ case reports, 2016, Jan-20, Volume: 2016

    Topics: Adult; Ammonia; Arginine; Female; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Phenylbutyrates; Sodium Benzoate; Treatment Outcome; Urea Cycle Disorders, Inborn

2016
Clinical phenotype, biochemical profile, and treatment in 19 patients with arginase 1 deficiency.
    Journal of inherited metabolic disease, 2016, Volume: 39, Issue:3

    Topics: Adolescent; Adult; Amino Acids; Arginase; Arginine; Case-Control Studies; Child; Child, Preschool; Female; Glycine; Humans; Infant; Male; Middle Aged; Nitric Oxide; Nitric Oxide Synthase Type II; Nitric Oxide Synthase Type III; Oxidative Stress; Phenotype; Retrospective Studies; Urea Cycle Disorders, Inborn; Young Adult

2016
Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001-2013.
    Orphanet journal of rare diseases, 2016, 08-19, Volume: 11, Issue:1

    Topics: Arginine; Child, Preschool; Female; Humans; Hyperammonemia; Infant; Kaplan-Meier Estimate; Liver Transplantation; Male; Ornithine Carbamoyltransferase Deficiency Disease; Prognosis; Retrospective Studies; Sodium Benzoate; Urea Cycle Disorders, Inborn

2016
Creatine metabolism in urea cycle defects.
    Journal of inherited metabolic disease, 2012, Volume: 35, Issue:4

    Topics: Adolescent; Adult; Amidinotransferases; Arginine; Child; Child, Preschool; Creatine; Female; Guanidinoacetate N-Methyltransferase; Humans; Infant; Language Development Disorders; Male; Movement Disorders; Nerve Tissue Proteins; Ornithine-Oxo-Acid Transaminase; Plasma Membrane Neurotransmitter Transport Proteins; Urea Cycle Disorders, Inborn; Young Adult

2012