ornithine has been researched along with Inborn Urea Cycle Disorder in 29 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 23 (79.31) | 24.3611 |
| 2020's | 6 (20.69) | 2.80 |
| Authors | Studies |
|---|---|
| Becerra, A; Gemperle, C; Gruppi, A; Häberle, J; Larovere, LE; Motrich, RD; Olivero, V; Peano, N; Silvera-Ruiz, SM | 1 |
| Bertini, E; Di Capua, M; Diodato, D; Dionisi-Vici, C; Longo, D; Martinelli, D; Olivieri, G; Pro, S | 1 |
| Agolini, E; Boenzi, S; Dionisi-Vici, C; Fiermonte, G; Goffredo, BM; Häberle, J; Martinelli, D; Porcelli, V; Travaglini, L | 1 |
| De Bruyne, P; De Bruyne, R; de Hemptinne, B; Van Biervliet, S; Van Hove, JLK; Van Winckel, M; Vande Velde, S; Verloo, P | 1 |
| Arnoux, JB; Besson, G; Bigot, A; Charriere, S; Damaj, L; Douillard, C; Garnotel, R; Kaphan, E; Lavigne, C; Maillot, F; Mesli, S; Mochel, F; Moreau, C; Noel, E; Odent, S; Redonnet-Vernhet, I; Rigalleau, V; Roubertie, A; Servais, A; Servettaz, A; Spodenkiewicz, M; Toquet, S | 1 |
| Billingham, MJ; Rizk, R | 1 |
| Fukuyama, T; Hoshino, Y; Kaneko, T; Kodaira, M; Matsuno, A; Sekijima, Y; Takano, K; Yazaki, M | 1 |
| Ono, H; Shigematsu, Y; Tamada, T | 1 |
| Enlund, M; Hårdstedt, M; Oscarson, M; Sahlander, F; Silfverberg, T | 1 |
| Barić, I; Burgard, P; Chapman, KA; Gleich, F; Kölker, S; Lund, AM; Molema, F; Summar, ML; van der Ploeg, AT; Williams, M | 1 |
| Busanello, EN; Grings, M; Leipnitz, G; Moura, AP; Tonin, AM; Viegas, CM; Wajner, M; Zanatta, A | 1 |
| Au, KM; Chan, AY; Lai, CK; Lai, JP; Lam, CW; Lee, HH; Mak, CM; Poon, KH; Siu, TS; Yuen, YP | 1 |
| Auray-Blais, C; Lavoie, P; Maranda, B | 1 |
| Agarwal, A; Chandani, N; Dhawan, A; Kumar, K; Raj, P | 1 |
| de Moura Coelho, D; de Oliveira Monteiro, W; Hickmann, FH; Leipnitz, G; Sitta, A; Vargas, CR; Viegas, CM; Wajner, M; Zanatta, Â | 1 |
| Calvello, R; Marobbio, CM; Palmieri, F; Palmieri, L; Panaro, MA; Pierri, CL; Punzi, G | 1 |
| Bertini, E; Boenzi, S; Diodato, D; Dionisi-Vici, C; Fiermonte, G; Martinelli, D; Monné, M; Ponzi, E | 1 |
| Cheillan, D; Dessein, AF; Dobbelaere, D; Fontaine, M; Joncquel-Chevalier Curt, M; Mention-Mulliez, K; Porchet, N; Soto-Ares, G; Vamecq, J; Voicu, PM | 1 |
| Lemire, EG; Mirhosseini, NZ; Moien-Afshari, F; Voll, CL | 1 |
| Amaral, AU; Quincozes-Santos, A; Rodrigues, MD; Souza, DG; Wajner, M; Zanatta, Â | 1 |
| Jamuar, SS; Lim, JS; Qadri, SK; Ting, TW | 1 |
| Antonishyn, N; Irvine, J; Lehotay, DC; Lepage, J; McDonald, R; Rockman-Greenberg, C; Sokoro, AA | 1 |
| Cianciara, J; Sikorska, H; Wiercińska-Drapało, A | 1 |
| Busanello, EN; de Moura, AP; Ferreira, Gda C; Grings, M; Ritter, L; Schuck, PF; Sitta, A; Tonin, AM; Vargas, CR; Viegas, CM; Wajner, M | 1 |
| Erez, A; Lee, B; Nagamani, SC | 1 |
| Hayasaka, S; Kodama, T; Ohira, A | 1 |
| Chou, KC; Wang, JF | 1 |
| Ficicioglu, C; Kim, SZ; Mandell, R; Nyhan, WL; Shih, VE; Song, WJ | 1 |
| Alberici, A; Filosto, M; Padovani, A; Santorelli, FM; Tessa, A | 1 |
6 review(s) available for ornithine and Inborn Urea Cycle Disorder
| Article | Year |
|---|---|
The hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Aging; Humans; Hyperammonemia; Mutation; Origin Recognition Complex; Ornithine; Protein Conformation; Urea Cycle Disorders, Inborn | 2015 |
Creatine biosynthesis and transport in health and disease.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; AMP-Activated Protein Kinases; Animals; Biological Transport, Active; Brain Diseases, Metabolic, Inborn; Creatine; Developmental Disabilities; Energy Metabolism; Guanidinoacetate N-Methyltransferase; Gyrate Atrophy; Humans; Hyperammonemia; Intellectual Disability; Language Development Disorders; Mental Retardation, X-Linked; Methylation; Mitochondrial Membrane Transport Proteins; Movement Disorders; Mutation; Nerve Tissue Proteins; Ornithine; Plasma Membrane Neurotransmitter Transport Proteins; Prenatal Diagnosis; S-Adenosylmethionine; Speech Disorders; Urea Cycle Disorders, Inborn | 2015 |
Milder Form of Urea Cycle Defect Revisited: Report and Review of Hyperornithinaemia-Hyperammonaemia-Homocitrullinuria (HHH) Syndrome Diagnosed in a Teenage Girl Presenting with Recurrent Encephalopathy.
Topics: Amino Acid Transport Systems, Basic; Brain Diseases; Child; Diet, Protein-Restricted; DNA Mutational Analysis; Female; Humans; Hyperammonemia; Mitochondrial Membrane Transport Proteins; Ornithine; Recurrence; Severity of Illness Index; Urea Cycle Disorders, Inborn | 2016 |
[Physiological functions of L-ornithine and L-aspartate in the body and the efficacy of administration of L-ornithine-L-aspartate in conditions of relative deficiency].
Topics: Animals; Aspartic Acid; Disease Models, Animal; Female; Humans; Ornithine; Physical Exertion; Pregnancy; Urea Cycle Disorders, Inborn; Wound Healing | 2010 |
Argininosuccinate lyase deficiency-argininosuccinic aciduria and beyond.
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 |
Retinal risks of high-dose ornithine supplements: a review.
Topics: Animals; Dietary Supplements; Dose-Response Relationship, Drug; Haplorhini; Heterozygote; Humans; Hyperammonemia; Mice; Models, Biological; Ornithine; Rats; Rats, Sprague-Dawley; Retina; Retinal Degeneration; Retinal Pigment Epithelium; Urea Cycle Disorders, Inborn | 2011 |
23 other study(ies) available for ornithine and Inborn Urea Cycle Disorder
| Article | Year |
|---|---|
Immune Alterations in a Patient With Hyperornithinemia-Hyperammonemia-Homocitrullinuria Syndrome: A Case Report.
Topics: Amino Acid Transport Systems, Basic; Child, Preschool; Female; Humans; Hyperammonemia; Mitochondrial Membrane Transport Proteins; Ornithine; Quality of Life; Urea Cycle Disorders, Inborn | 2022 |
Corticospinal tract damage in HHH syndrome: a metabolic cause of hereditary spastic paraplegia.
Topics: Adolescent; Adult; Brain; Child; Female; Humans; Hyperammonemia; Magnetic Resonance Imaging; Male; Middle Aged; Mutation; Neural Conduction; Ornithine; Spastic Paraplegia, Hereditary; Spinal Cord; Urea Cycle Disorders, Inborn; Young Adult | 2019 |
CUGC for hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome.
Topics: Genetic Testing; Humans; Hyperammonemia; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Phenotype; Sensitivity and Specificity; Urea Cycle Disorders, Inborn | 2020 |
Successful liver transplantation in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: Case report.
Topics: Child; Humans; Hyperammonemia; Liver Transplantation; Male; Ornithine; Urea Cycle Disorders, Inborn | 2021 |
Adult-onset diagnosis of urea cycle disorders: Results of a French cohort of 71 patients.
Topics: Adolescent; Adult; Age of Onset; Aged; Aged, 80 and over; Argininosuccinic Aciduria; Female; France; Humans; Hyperammonemia; Male; Middle Aged; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Retrospective Studies; Sex Factors; Urea Cycle Disorders, Inborn; Young Adult | 2021 |
Role of early management of hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome in pregnancy.
Topics: Citrulline; Female; Humans; Hyperammonemia; Ornithine; Pregnancy; Urea Cycle Disorders, Inborn | 2021 |
Reversible Leukoencephalopathy in a Man with Childhood-onset Hyperornithinemia-Hyperammonemia-Homocitrullinuria Syndrome.
Topics: Ammonia; Child; Humans; Hyperammonemia; Leukoencephalopathies; Male; Middle Aged; Ornithine; Urea Cycle Disorders, Inborn | 2022 |
Lactate/pyruvate in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Arginine; Diet, Protein-Restricted; Humans; Hyperammonemia; Infant; Lactates; Male; Ornithine; Pyruvates; Urea Cycle Disorders, Inborn | 2018 |
Late onset hyperornithinemia-hyperammonemia-homocitrullinuria syndrome - how web searching by the family solved unexplained unconsciousness: a case report.
Topics: Female; Humans; Hyperammonemia; Internet; Male; Middle Aged; Ornithine; Unconsciousness; Urea Cycle Disorders, Inborn | 2018 |
Evaluation of dietary treatment and amino acid supplementation in organic acidurias and urea-cycle disorders: On the basis of information from a European multicenter registry.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Child; Child, Preschool; Cross-Sectional Studies; Dietary Supplements; Europe; Feasibility Studies; Female; Humans; Hyperammonemia; Infant; Male; Ornithine; Propionic Acidemia; Registries; Retrospective Studies; Treatment Outcome; Urea Cycle Disorders, Inborn; Young Adult | 2019 |
Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: a possible mechanism of cerebellar dysfunction in HHH syndrome.
Topics: Aconitate Hydratase; Animals; Cerebellum; Citrulline; Creatine Kinase; Electron Transport; Glutathione; Homeostasis; Hydrogen Peroxide; Hyperammonemia; Ketoglutarate Dehydrogenase Complex; Nerve Tissue Proteins; Nitrates; Nitrites; Ornithine; Oxidation-Reduction; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Thiobarbituric Acid Reactive Substances; Urea Cycle Disorders, Inborn | 2013 |
Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome: a treatable genetic liver disease warranting urgent diagnosis.
Topics: Amino Acid Transport Systems, Basic; Amino Acids; Child; Child, Preschool; Heterozygote; Humans; Hyperammonemia; Infant; Infant, Newborn; Male; Mitochondrial Membrane Transport Proteins; Neonatal Screening; Ornithine; Prenatal Diagnosis; Urea Cycle Disorders, Inborn | 2014 |
High-throughput tandem mass spectrometry multiplex analysis for newborn urinary screening of creatine synthesis and transport disorders, Triple H syndrome and OTC deficiency.
Topics: Biomarkers; Creatine; Creatinine; Filtration; Glycine; Humans; Hyperammonemia; Infant, Newborn; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Paper; Reference Values; Reproducibility of Results; Tandem Mass Spectrometry; Uracil; Urea Cycle Disorders, Inborn; Urinalysis | 2014 |
Ocular manifestations in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: a rare association.
Topics: Adolescent; Diagnosis, Differential; Fluorescein Angiography; Fovea Centralis; Fundus Oculi; Humans; Hyperammonemia; Male; Myopia; Ornithine; Retinal Degeneration; Tomography, Optical Coherence; Urea Cycle Disorders, Inborn; Vitreous Body | 2015 |
Ornithine In Vivo Administration Disrupts Redox Homeostasis and Decreases Synaptic Na(+), K (+)-ATPase Activity in Cerebellum of Adolescent Rats: Implications for the Pathogenesis of Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) Syndrome.
Topics: Animals; Antioxidants; Cerebellum; Glutathione; Homeostasis; Hyperammonemia; Lipid Peroxidation; Male; Malondialdehyde; Ornithine; Oxidation-Reduction; Rats; Rats, Wistar; Sexual Maturation; Sodium-Potassium-Exchanging ATPase; Synapses; Urea Cycle Disorders, Inborn | 2015 |
Pathogenic potential of SLC25A15 mutations assessed by transport assays and complementation of Saccharomyces cerevisiae ORT1 null mutant.
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 |
An Acute Encephalopathy Accelerated by a Large Amount of Milk Consumption.
Topics: Adult; Animals; Drinking; Humans; Hyperammonemia; Male; Milk; Native Hawaiian or Other Pacific Islander; Ornithine; Urea Cycle Disorders, Inborn | 2016 |
Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome.
Topics: Amino Acid Transport Systems, Basic; Animals; Antioxidants; Astrocytes; Cell Death; Citrulline; Hyperammonemia; Male; Mitochondria; Ornithine; Rats, Wistar; Tumor Necrosis Factor-alpha; Urea Cycle Disorders, Inborn | 2016 |
Diagnosis and high incidence of hyperornithinemia-hyperammonemia-homocitrullinemia (HHH) syndrome in northern Saskatchewan.
Topics: Amino Acid Transport Systems, Basic; Biomarkers; DNA Mutational Analysis; Dried Blood Spot Testing; Gene Frequency; Genetic Predisposition to Disease; Genetic Testing; Heterozygote; Homozygote; Humans; Hyperammonemia; Incidence; Infant, Newborn; Mitochondrial Membrane Transport Proteins; Mutation; Neonatal Screening; Ornithine; Phenotype; Polymerase Chain Reaction; Predictive Value of Tests; Prospective Studies; Retrospective Studies; Saskatchewan; Tandem Mass Spectrometry; Time Factors; Urea Cycle Disorders, Inborn | 2010 |
Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Animals; Cerebral Cortex; Citrulline; Hyperammonemia; Injections, Intraventricular; Lipid Peroxidation; Ornithine; Oxidative Stress; Rats; Rats, Wistar; Urea Cycle Disorders, Inborn | 2011 |
Insights into the mutation-induced HHH syndrome from modeling human mitochondrial ornithine transporter-1.
Topics: Amino Acid Sequence; Amino Acid Transport Systems, Basic; Binding Sites; Computer Simulation; Humans; Hyperammonemia; Mitochondrial Membrane Transport Proteins; Models, Molecular; Molecular Dynamics Simulation; Molecular Sequence Data; Mutation; Ornithine; Protein Binding; Protein Interaction Domains and Motifs; Protein Interaction Mapping; Sequence Homology, Amino Acid; Urea Cycle Disorders, Inborn | 2012 |
Long-term follow-up of four patients affected by HHH syndrome.
Topics: Adult; Amino Acid Transport Systems, Basic; Amino Acids; Chromatography, High Pressure Liquid; Chromatography, Ion Exchange; Disease Progression; DNA Mutational Analysis; Female; Follow-Up Studies; Humans; Hyperammonemia; Male; Middle Aged; Mitochondrial Membrane Transport Proteins; Ornithine; Phenotype; Time Factors; Urea Cycle Disorders, Inborn | 2012 |
Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome in adulthood: a rare recognizable condition.
Topics: Adult; Amino Acid Sequence; Amino Acid Transport Systems, Basic; Base Sequence; Humans; Hyperammonemia; Male; Mitochondrial Membrane Transport Proteins; Molecular Sequence Data; Mutation; Ornithine; Urea Cycle Disorders, Inborn | 2013 |