Page last updated: 2024-08-24

homocitrulline and ornithine

homocitrulline has been researched along with ornithine in 63 studies

Research

Studies (63)

Timeframe	Studies, this research(%)	All Research%
pre-1990	25 (39.68)	18.7374
1990's	12 (19.05)	18.2507
2000's	19 (30.16)	29.6817
2010's	7 (11.11)	24.3611
2020's	0 (0.00)	2.80

Authors

Authors	Studies
Gatfield, PD; Haust, MD; Taller, E; Wolfe, DM	1
Goto, I; Kobayashi, T; Shigeto, H; Yamada, T	1
Shimizu, H	1
Arbour, JF; Dubé, J; Flessas, J; Laberge, M; Lafleur, L; Lambert, MA; Lemay, JF; Mitchell, GA; Valle, D; Vanasse, M	1
Laframboise, R; Mandell, R; Pichette, J; Shih, VE	1
Brochu, P; Jasmin, G; Lambert, MA; Qureshi, IA; Seidman, EG; Smith, L	1
Azuma, T; Higa, S; Kishimoto, S; Sakoda, S; Suzuki, T; Tsujino, S	1
Knopman, DS; Shih, VE; Tuchman, M	1
Kato, T; Mizutani, N; Sano, M	1
Botschner, J; Scriver, CR; Simell, O; Smith, DW	1
Bonnefont, JP; Chadefaux, B; Kamoun, P; Rabier, D; Saudubray, JM; Shih, VE	1
Alme, A; Broch, H; Gjessing, LR; Lie, SO; Lunde, HA; Undrum, T	1
Alvarez, L; Coude, FX; Fabregas, I; Fernandez Alvarez, E; Grimber, G; Pineda, M; Ribes, A; Rodes, M	1
Bachmann, C; Colombo, JP; Dionisi Vici, C; Gambarara, M; Sabetta, G	1
Oyanagi, K	1
Inoue, I; Kayanuma, K; Koike, R; Miyatake, T; Nakajima, M; Saheki, T; Sakoda, K; Takeshita, K; Uono, M; Yuasa, T	1
Hashiba, M; Noguchi, R; Shimoji, K; Tomita, Y	1
Akiyoshi, H; Ichihara, K; Inoue, I; Ishii, S; Mito, T; Nakajima, M; Saheki, T; Takakura, H; Takashima, S; Takeshita, K	1
Fujimori, K; Koike, R; Miyatake, T; Yuasa, T	1
Gatfield, DP; Gordon, BA; Haust, MD	1
Biscoping, J; Hempelmann, G; Michaelis, G	1
Dyken, PR; Hartlage, PL; Hommes, FA; Metoki, K; Roesel, RA	1
Clow, CL; Mackenzie, S; Scriver, CR; Simell, O	1
Gray, RG; Hill, SE; Pollitt, RJ	1
Itakura, Y; Nakao, T; Oyanagi, K; Sakamoto, S; Sogawa, H; Tsuchiyama, A; Wagatsuma, K; Yachi, A	1
Hommes, FA; Metoki, K	1
Carter, AL; Eller, AG; Hommes, FA; Metoki, K; Rufo, S	1
Dyken, P; Hommes, FA; Kelloes, C; Metoki, K; Trefz, J	1
Carter, AL; Eller, AG; Evans, BA; Hommes, FA	1
Shih, VE	1
Herzfeld, A; Mandell, R; Shih, VE	1
Gordon, BA; Haust, MD	1
Gray, RG; Green, A; Hall, S; McKeown, C	1
Donati, MA; Filippi, L; Resti, M; Zammarchi, E	1
Bonocore, G; Buonocore, G; Ciani, F; Donati, MA; Pasquini, E; Shih, VE; Zammarchi, E	1
Nagao, M; Oyanagi, K	1
Celli, M; Crifò, C; D'Eufemia, P; Finocchiaro, R; Giardini, O; Salerno, C	1
Gallagher, AC; Pike, M; Standing, S	1
Ohura, T; Sakamoto, O	1
Bertini, E; Boldrini, R; Burlina, AB; Di Capua, M; Dionisi-Vici, C; Donati, A; Fariello, G; Meli, C; Rizzo, C; Salvi, S; Santorelli, FM	1
Bertini, E; Dionisi-Vici, C; Salvi, S; Santorelli, FM; Verardo, M	1
Dehne, MG; Fuchs, M; Hempelmann, G; Mühling, J; Sablotzki, A; Spatz, J; Weiss, S	1
Kakinuma, H	1
Kanazawa, N; Miyamoto, T; Tsujino, S	1
David, L; Dionisi-Vici, C; Dolce, V; Fiermonte, G; Palmieri, F; Santorelli, FM; Walker, JE	1
Andrade, D; Camacho, JA; Kong, J; Porter, J; Rioseco-Camacho, N	1
Abu-Libdeh, B; Gutman, A; Kanazawa, N; Korman, SH; Tsujino, S	1
Andria, G; Carbone, MT; Correra, A; Della Casa, R; Dionisi-Vici, C; Fecarotta, S; Parenti, G; Riva, S; Santorelli, FM; Torre, G; Vajro, P; Zuppaldi, A	1
Al-Dirbashi, OY; Al-Hassnan, ZN; Rashed, MS	1
Abu-Amero, KK; Al-Dirbashi, OY; Al-Hassnan, ZN; Patay, Z; Rahbeeni, Z; Rashed, MS	1
Chan, A; Collison, M; Greenberg, CR; Huynh, HQ; Lehotay, DC; Mhanni, AA; Seifert, B; Sokoro, A	1
Debray, FG; Drouin, R; Dubé, J; Fenyves, D; Laframboise, R; Lambert, M; Lemieux, B; Maranda, B; Mitchell, GA; Soucy, JF	1
Aldamiz-Echevarría, L; Baumgartner, MR; Besley, G; Chien, YH; de Baulny, HO; Deodato, F; Dionisi-Vici, C; Fiermonte, G; Hernandez, JM; Loguercio, C; Martinez-Hernandez, E; Melone, MA; Nassogne, MC; Palmieri, F; Paradies, E; Parenti, G; Pierri, CL; Rutledge, SL; Santorelli, FM; Scarano, G; Schiff, M; Tessa, A; Vilaseca, MA; Walter, J	1
Amaral, AU; Dutra Filho, CS; Ferreira, Gda C; Knebel, LA; Schuck, PF; Tonin, AM; Viegas, CM; Wajner, M; Wannmacher, CM; Zanatta, A	1
Boenzi, S; Capozzi, P; Dionisi-Vici, C; Morini, C; Rizzo, C; Santorelli, FM	1
Amaral, AU; Dutra-Filho, CS; Fernandes, CG; Leipnitz, G; Seminotti, B; Viegas, CM; Wajner, M; Zanatta, 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
Busanello, EN; Grings, M; Knebel, LA; Leipnitz, G; Lobato, VA; Moura, AP; Pettenuzzo, LF; Ritter, L; Tonin, AM; Vargas, CR; Viegas, CM; Wajner, M; Zanatta, A	1
Busanello, EN; Fernandes, CG; Leipnitz, G; Moura, AP; Seminotti, B; Tonin, AM; Viegas, CM; Wajner, M; Zanatta, A	1
Busanello, EN; Grings, M; Leipnitz, G; Moura, AP; Tonin, AM; Viegas, CM; Wajner, M; Zanatta, A	1
Amaral, AU; Quincozes-Santos, A; Rodrigues, MD; Souza, DG; Wajner, M; Zanatta, Â	1
Alten, R; Bang, H; Cobra, JF; Englbrecht, M; Feuchtenberger, M; Figueiredo, CP; Finzel, S; Fleck, M; Haschka, J; Henes, J; Hueber, AJ; Kleinert, S; Kleyer, A; Krueger, K; Lorenz, HM; Manger, B; Manger, K; Nuesslein, H; Ochs, W; Rech, J; Reiser, M; Ronneberger, M; Schett, G; Schmitt-Haendle, M; Schuch, F; Tony, HP; Wendler, J	1
Becker, T; Hanff, E; Tsikas, D	1

Reviews

6 review(s) available for homocitrulline and ornithine

Article	Year
Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome: presentation as acute liver disease with coagulopathy. Journal of pediatric gastroenterology and nutrition, 1992, Volume: 15, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Blood Coagulation Disorders; Child, Preschool; Citrulline; Female; Humans; Liver Diseases; Liver Function Tests; Ornithine; Syndrome	1992
Regulation of ornithine metabolism. Enzyme, 1981, Volume: 26, Issue:5 Topics: Ammonia; Animals; Biological Transport; Citrulline; Cytosol; Fibroblasts; Humans; Mitochondria; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase; Urea	1981
[Hyperornithinemia-hyperammonemia-homocitrullinuria (H.H.H.) syndrome]. Ryoikibetsu shokogun shirizu, 1998, Issue:18 Pt 1 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Diagnosis, Differential; Humans; Infant, Newborn; Ornithine; Ornithine-Oxo-Acid Transaminase; Syndrome	1998
[Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome]. Ryoikibetsu shokogun shirizu, 2001, Issue:33 Topics: Citrulline; Humans; Hyperammonemia; Ornithine; Syndrome	2001
[Structure-function relationships of mitochondrial transporters]. Nihon rinsho. Japanese journal of clinical medicine, 2002, Volume: 60 Suppl 4 Topics: Animals; Citrulline; Humans; Hyperammonemia; Intracellular Membranes; Membrane Transport Proteins; Mitochondria; Mitochondrial ADP, ATP Translocases; Mutation; Ornithine; Phosphate Transport Proteins; Syndrome	2002
[Mitochondrial ornithine transporter deficiency]. Nihon rinsho. Japanese journal of clinical medicine, 2002, Volume: 60 Suppl 4 Topics: Amino Acid Transport Systems, Basic; Carrier Proteins; Citrulline; Diagnosis, Differential; Humans; Hyperammonemia; Membrane Transport Proteins; Mitochondria; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Prognosis; Proteins; Syndrome	2002

Other Studies

57 other study(ies) available for homocitrulline and ornithine

Article	Year
Hyperornithinemia, hyperammonemia, and homocitrullinuria associated with decreased carbamyl phosphate synthetase I activity. Pediatric research, 1975, Volume: 9, Issue:5 Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Biopsy; Carbamoyl-Phosphate Synthase (Ammonia); Child; Child, Preschool; Citrulline; Dietary Proteins; Female; Humans; Leukocytes; Liver; Lysine; Male; Microscopy, Electron; Mitochondria, Liver; Ornithine; Phosphotransferases	1975
[A case of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs]. Rinsho shinkeigaku = Clinical neurology, 1992, Volume: 32, Issue:7 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Electromyography; Humans; Leg; Male; Muscular Atrophy; Ornithine; Paraparesis, Tropical Spastic; Spinal Cord; Syndrome	1992
[The dibasic amino acid metabolic disorders]. Nihon rinsho. Japanese journal of clinical medicine, 1992, Volume: 50, Issue:7 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Blood Transfusion; Citrulline; Humans; Hyperargininemia; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Renal Dialysis	1992
Hyperammonemia-hyperornithinemia-homocitrullinuria syndrome: neurologic, ophthalmologic, and neuropsychologic examination of six patients. The Journal of pediatrics, 1992, Volume: 121, Issue:5 Pt 1 Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Child, Preschool; Citrulline; Eye Diseases; Female; Humans; Male; Nervous System Diseases; Neuropsychological Tests; Ornithine; Psychomotor Performance	1992
Neonatal form of the hyperornithinaemia, hyperammonaemia, and homocitrullinuria (HHH) syndrome and prenatal diagnosis. Prenatal diagnosis, 1992, Volume: 12, Issue:9 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Cells, Cultured; Citrulline; Female; Gestational Age; Hepatitis B Surface Antigens; Humans; Hyaline Membrane Disease; Infant, Newborn; Male; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Ornithine-Oxo-Acid Transaminase; Pregnancy; Pregnancy Outcome; Prenatal Diagnosis; Syndrome	1992
Hyperornithinemia, hyperammonemia and homocitrullinuria--a case report and study of ornithine metabolism using in vivo deuterium labelling. Clinica chimica acta; international journal of clinical chemistry, 1991, Sep-14, Volume: 201, Issue:1-2 Topics: Adult; Ammonia; Arginine; Chromatography, Liquid; Citrulline; Deuterium; Humans; Male; Mass Spectrometry; Ornithine	1991
Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Archives of neurology, 1990, Volume: 47, Issue:10 Topics: Adult; Ammonia; Citrulline; Female; Fibroblasts; Glutamine; Humans; Leucine; Male; Metabolism, Inborn Errors; Ornithine; Phenylbutyrates; Recurrence; Syndrome; Urea	1990
Homocitrullinuria and homoargininuria in lysinuric protein intolerance. Journal of inherited metabolic disease, 1989, Volume: 12, Issue:2 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Arginine; Child; Citrulline; Female; Homoarginine; Humans; Kidney; Lysine; Male; Ornithine	1989
Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts. Journal of inherited metabolic disease, 1989, Volume: 12, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Fibroblasts; Heterozygote; Homozygote; Humans; Lysine; Ornithine; Ornithine-Oxo-Acid Transaminase; Oxidation-Reduction; Proteins; Syndrome	1989
Potential for the prenatal diagnosis of hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. American journal of medical genetics, 1989, Volume: 32, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Chorionic Villi Sampling; Citrulline; Female; Humans; Ornithine; Pregnancy; Pregnancy Trimester, First; Prenatal Diagnosis	1989
A new patient with hyperornithinaemia, hyperammonaemia and homocitrullinuria treated early with low protein diet. Journal of inherited metabolic disease, 1986, Volume: 9, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Citrulline; Dietary Proteins; Glutamine; Humans; Infant; Male; Ornithine; Pyrimidines	1986
A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria. Journal of inherited metabolic disease, 1987, Volume: 10, Issue:1 Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Citrulline; Female; Humans; Male; Ornithine; Syndrome	1987
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement. Pediatric research, 1987, Volume: 22, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Child, Preschool; Citrulline; Creatine; Female; Humans; Lysine; Male; Ornithine; Orotic Acid; Syndrome	1987
[Hyperornithinemia, hyperammonemia and homocitrullinuria due to a defect of mitochondrial membrane transport]. Tanpakushitsu kakusan koso. Protein, nucleic acid, enzyme, 1988, Volume: 33, Issue:5 Topics: Amino Acids; Ammonia; Biological Transport; Cells, Cultured; Citrulline; Fibroblasts; Humans; Mitochondria, Liver; Ornithine; Skin	1988
Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria. Biochimica et biophysica acta, 1988, Jan-12, Volume: 964, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Biological Transport; Citrulline; Humans; Kinetics; Mitochondria, Liver; Ornithine	1988
[Anesthesia for a patient with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome]. Masui. The Japanese journal of anesthesiology, 1988, Volume: 37, Issue:1 Topics: Adult; Ammonia; Anesthesia; Citrulline; Humans; Male; Ornithine; Syndrome	1988
Clinical, biochemical and ultrastructural study on the pathogenesis of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Brain & development, 1988, Volume: 10, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Brain; Child; Citrulline; Endoplasmic Reticulum; Humans; Liver Glycogen; Male; Mitochondria, Liver; Ornithine; Tomography, X-Ray Computed	1988
[Hyperornithinemia, hyperammonemia and homocitrullinuria syndrome in a family]. Rinsho shinkeigaku = Clinical neurology, 1987, Volume: 27, Issue:4 Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Electroencephalography; Humans; Male; Mitochondria; Ornithine; Syndrome	1987
The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements. Clinical and investigative medicine. Medecine clinique et experimentale, 1987, Volume: 10, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Citrulline; Dietary Proteins; Fibroblasts; Humans; In Vitro Techniques; Male; Mitochondria; Ornithine; Oxidation-Reduction; Syndrome	1987
[H-H-H syndrome: a rare disease also relevant in anesthesiology]. Anasthesie, Intensivtherapie, Notfallmedizin, 1986, Volume: 21, Issue:6 Topics: Adolescent; Ammonia; Anesthesia, General; Citrulline; Femoral Fractures; Fracture Fixation, Internal; Humans; Male; Ornithine; Postoperative Complications; Syndrome; Wound Healing	1986
Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria). Neuropediatrics, 1986, Volume: 17, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Biological Transport; Child, Preschool; Citrulline; Fibroblasts; Follow-Up Studies; Humans; Kinetics; Male; Ornithine; Psychomotor Disorders; Seizures; Syndrome	1986
Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Pediatric research, 1985, Volume: 19, Issue:12 Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Child; Citrulline; Cytoplasm; Humans; Male; Ornithine; Orotic Acid; Syndrome	1985
Reduced ornithine catabolism in cultured fibroblasts and phytohaemagglutinin-stimulated lymphocytes from a patient with hyperornithinaemia, hyperammonaemia and homocitrullinuria. Clinica chimica acta; international journal of clinical chemistry, 1982, Feb-05, Volume: 118, Issue:2-3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Aspartate Ammonia-Lyase; Cells, Cultured; Citrulline; Fibroblasts; Glutamates; Glutamic Acid; Humans; Lymphocyte Activation; Lymphocytes; Ornithine; Phytohemagglutinins; Proline	1982
The mechanism of hyperammonaemia and hyperornithinaemia in the syndrome of hyperornithinaemia, hyperammonaemia with homocitrullinuria. Journal of inherited metabolic disease, 1983, Volume: 6, Issue:3 Topics: Ammonia; Citrulline; Fibroblasts; Humans; Liver; Male; Middle Aged; Mitochondria, Liver; Ornithine; Syndrome	1983
The pH of mitochondria of fibroblasts from a hyperornithinaemia, hyperammonaemia, homocitrullinuria-syndrome patient. Journal of inherited metabolic disease, 1984, Volume: 7, Issue:1 Topics: Acid-Base Equilibrium; Ammonia; Citrulline; Cytosol; Fibroblasts; Humans; Hydrogen-Ion Concentration; Mitochondria; Ornithine; Syndrome	1984
Further evidence for a separate enzymic entity for the synthesis of homocitrulline, distinct from the regular ornithine transcarbamylase. Enzyme, 1984, Volume: 32, Issue:1 Topics: Animals; Citrulline; Female; Hydrogen-Ion Concentration; Kinetics; Lysine; Male; Mice; Mice, Mutant Strains; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase; Rats	1984
Ultrastructural changes in fibroblast mitochondria of a patient with HHH-syndrome. Journal of inherited metabolic disease, 1984, Volume: 7, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Fibroblasts; Genes, Recessive; Humans; Intracellular Membranes; Microscopy, Electron; Mitochondria; Ornithine; Skin	1984
Reconstitution of ornithine transport in liposomes with Lubrol extracts of mitochondria. FEBS letters, 1984, May-07, Volume: 170, Issue:1 Topics: Amino Acid Transport Systems, Basic; Ammonia; Animals; Biological Transport, Active; Carrier Proteins; Cattle; Citrulline; Kinetics; Liposomes; Membrane Transport Proteins; Mitochondria, Liver; Ornithine; Polyethylene Glycols; Solubility; Syndrome	1984
Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria. Clinica chimica acta; international journal of clinical chemistry, 1982, Feb-05, Volume: 118, Issue:2-3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Cells, Cultured; Citrulline; Fibroblasts; Humans; Models, Chemical; Ornithine; Proteins; Skin	1982
Ultrastructural changes in the mitochondria in disorders in ornithine metabolism. Pediatric research, 1980, Volume: 14, Issue:12 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Humans; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease	1980
Prenatal exclusion of the HHH syndrome. Prenatal diagnosis, 1995, Volume: 15, Issue:5 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Amniotic Fluid; Cells, Cultured; Citrulline; Female; Humans; Ornithine; Pregnancy; Prenatal Diagnosis; Syndrome	1995
Cryptogenic hepatitis masking the diagnosis of ornithine transcarbamylase deficiency. Journal of pediatric gastroenterology and nutrition, 1996, Volume: 22, Issue:4 Topics: Absorption; Ammonia; Arginine; Child, Preschool; Citrulline; Diagnosis, Differential; Dietary Proteins; Female; Glutamine; Hepatitis; Humans; Infant; Lysine; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Urea	1996
Neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with favorable outcome. The Journal of pediatrics, 1997, Volume: 131, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Citrulline; Diet, Protein-Restricted; Humans; Infant, Newborn; Male; Ornithine	1997
Determination of urinary orotic acid and uracil by capillary zone electrophoresis. Journal of chromatography. B, Biomedical sciences and applications, 1999, Oct-29, Volume: 734, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Borates; Buffers; Citrulline; Dietary Proteins; Electrophoresis, Capillary; Humans; Hydrogen-Ion Concentration; Infant; Male; Ornithine; Orotic Acid; Syndrome; Uracil	1999
HHH syndrome associated with callosal agenesis and disordered neuronal migration. Developmental medicine and child neurology, 2001, Volume: 43, Issue:6 Topics: Abnormalities, Multiple; Agenesis of Corpus Callosum; Bronchopulmonary Sequestration; Citrulline; Consanguinity; Female; Humans; Hyperammonemia; Infant; Metabolism, Inborn Errors; Ornithine; Phenotype; Syndrome	2001
Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Neurology, 2001, Sep-11, Volume: 57, Issue:5 Topics: Adolescent; Adult; Brain Diseases, Metabolic, Inborn; Child; Citrulline; Female; Humans; Hyperammonemia; Male; Mitochondria, Muscle; Mutation; Ornithine; Paraparesis, Spastic; Retrospective Studies	2001
Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Human mutation, 2001, Volume: 18, Issue:5 Topics: Age of Onset; Alleles; Amino Acid Transport Systems, Basic; Base Sequence; Citrulline; DNA Mutational Analysis; Exons; Female; Fibroblasts; Humans; Hyperammonemia; Infant, Newborn; Italy; Male; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Pedigree; Polymorphism, Restriction Fragment Length; Proteins; Reverse Transcriptase Polymerase Chain Reaction; Syndrome	2001
Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia. Amino acids, 2001, Volume: 21, Issue:3 Topics: Adult; Amino Acids; Ammonia; Anesthesia; Blood Coagulation Tests; Citrulline; Clinical Chemistry Tests; Female; Humans; Monitoring, Physiologic; Ornithine; Syndrome	2001
The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms. The Journal of biological chemistry, 2003, Aug-29, Volume: 278, Issue:35 Topics: Amino Acid Transport Systems, Basic; Arginine; Biological Transport; Carrier Proteins; Citrulline; DNA, Complementary; Electrophoresis, Polyacrylamide Gel; Humans; Hyperammonemia; Kinetics; Liposomes; Liver; Lysine; Malates; Membrane Transport Proteins; Mitochondria; Models, Biological; Mutation; Ornithine; Phosphates; Protein Folding; Protein Isoforms; Recombinant Proteins; Reverse Transcriptase Polymerase Chain Reaction; Substrate Specificity; Time Factors; Tissue Distribution; Transcription, Genetic	2003
Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder. Molecular genetics and metabolism, 2003, Volume: 79, Issue:4 Topics: Amino Acid Sequence; Amino Acid Transport Systems, Basic; Cell Line; Citrulline; Cloning, Molecular; Fibroblasts; Gene Frequency; Humans; Hyperammonemia; Membrane Transport Proteins; Mitochondrial Membrane Transport Proteins; Molecular Sequence Data; Ornithine; Polymorphism, Genetic; Proteins; Syndrome	2003
Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family. Journal of the neurological sciences, 2004, Mar-15, Volume: 218, Issue:1-2 Topics: Adolescent; Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Arabs; Child, Preschool; Citrulline; DNA Mutational Analysis; Exons; Family Health; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Hyperammonemia; Lactic Acid; Male; Mitochondrial Diseases; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Polymerase Chain Reaction; Proteins	2004
HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation. Journal of inherited metabolic disease, 2006, Volume: 29, Issue:1 Topics: Alanine Transaminase; Amino Acid Transport Systems, Basic; Arginine; Aspartate Aminotransferases; Child, Preschool; Citrulline; Diet, Protein-Restricted; Hepatitis; Humans; Hyperammonemia; Liver; Male; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Proteins; Syndrome	2006
Determination of homocitrulline in urine of patients with HHH syndrome by liquid chromatography tandem mass spectrometry. Analytical and bioanalytical chemistry, 2006, Volume: 386, Issue:7-8 Topics: Ammonia; Chromatography, Liquid; Citrulline; Creatinine; Humans; Mass Spectrometry; Ornithine; Syndrome	2006
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with stroke-like imaging presentation: clinical, biochemical and molecular analysis. Journal of the neurological sciences, 2008, Jan-15, Volume: 264, Issue:1-2 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Brain; Brain Diseases, Metabolic, Inborn; Child; Child, Preschool; Citrulline; DNA Mutational Analysis; Female; Food, Formulated; Genetic Markers; Genetic Predisposition to Disease; Genetic Testing; Genotype; Humans; Hyperammonemia; Magnetic Resonance Imaging; Male; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Pedigree; Saudi Arabia; Sodium Benzoate; Stroke	2008
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) presenting with acute fulminant hepatic failure. Journal of pediatric gastroenterology and nutrition, 2008, Volume: 46, Issue:3 Topics: Amino Acid Transport Systems, Basic; Citrulline; Diet, Protein-Restricted; Female; Humans; Hyperammonemia; Infant; Liver Failure, Acute; Liver Transplantation; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Point Mutation; Syndrome	2008
Phenotypic variability among patients with hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome homozygous for the delF188 mutation in SLC25A15. Journal of medical genetics, 2008, Volume: 45, Issue:11 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Child; Child, Preschool; Citrulline; Founder Effect; Homozygote; Humans; Hyperammonemia; Infant; Mutation; Ornithine; Phenotype; Syndrome	2008
Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study. Human mutation, 2009, Volume: 30, Issue:5 Topics: Adult; Amino Acid Transport Systems, Basic; Biological Transport; Child; Child, Preschool; Citrulline; Escherichia coli; Female; Humans; Hyperammonemia; Infant; Infant, Newborn; Male; Middle Aged; Mitochondrial Membrane Transport Proteins; Mutant Proteins; Mutation; Ornithine; Protein Structure, Secondary; Syndrome	2009
Experimental evidence that ornithine and homocitrulline disrupt energy metabolism in brain of young rats. Brain research, 2009, Sep-29, Volume: 1291 Topics: Analysis of Variance; Animals; Cerebral Cortex; Citric Acid Cycle; Citrulline; Creatine Kinase; Cytosol; Electron Transport Chain Complex Proteins; In Vitro Techniques; Mitochondria; Ornithine; Orotic Acid; Oxygen Consumption; Rats; Sodium-Potassium-Exchanging ATPase; Spectrophotometry; Synaptic Membranes	2009
Retinal degeneration. Ophthalmology, 2009, Volume: 116, Issue:8 Topics: Adult; Amino Acid Transport Systems, Basic; Cataract; Citrulline; Consanguinity; Creatine; Diet, Protein-Restricted; Female; Humans; Hyperammonemia; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Retinal Degeneration; Syndrome; Vision Disorders	2009
Evidence that the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome induce oxidative stress in brain of young rats. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2009, Volume: 27, Issue:7 Topics: Animals; Antioxidants; Brain; Citrulline; Glutathione; Humans; Hyperammonemia; Lipid Peroxidation; Male; Nitric Oxide; Ornithine; Oxidation-Reduction; Oxidative Stress; Rats; Rats, Wistar; Syndrome; Thiobarbituric Acid Reactive Substances	2009
Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Brain research, 2011, Jan-19, Volume: 1369 Topics: Animals; Cerebral Cortex; Citrulline; Hyperammonemia; Injections, Intraventricular; Lipid Peroxidation; Ornithine; Oxidative Stress; Rats; Rats, Wistar; Urea Cycle Disorders, Inborn	2011
Chronic postnatal ornithine administration to rats provokes learning deficit in the open field task. Metabolic brain disease, 2012, Volume: 27, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Animals, Newborn; Avoidance Learning; Behavior, Animal; Citrulline; Cognition; Developmental Disabilities; Disease Models, Animal; Half-Life; Learning Disabilities; Maze Learning; Memory; Memory, Long-Term; Ornithine; Postural Balance; Rats; Rats, Wistar; Thiobarbituric Acid Reactive Substances	2012
Impairment of brain redox homeostasis caused by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome in vivo. Metabolic brain disease, 2012, Volume: 27, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Antioxidants; Brain; Catalase; Citrulline; Glutathione Peroxidase; Homeostasis; Injections, Intraperitoneal; Injections, Intraventricular; Male; Nitric Oxide; Ornithine; Oxidation-Reduction; Oxidative Stress; Protein Carbonylation; Rats; Rats, Wistar; Sulfhydryl Compounds; Thiobarbituric Acid Reactive Substances; Urease	2012
Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: a possible mechanism of cerebellar dysfunction in HHH syndrome. Life sciences, 2013, Aug-06, Volume: 93, Issue:4 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
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. Neurochemical research, 2016, Volume: 41, Issue:9 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
Antimodified protein antibody response pattern influences the risk for disease relapse in patients with rheumatoid arthritis tapering disease modifying antirheumatic drugs. Annals of the rheumatic diseases, 2017, Volume: 76, Issue:2 Topics: Acetates; Acetylation; Antirheumatic Agents; Arthritis, Rheumatoid; Autoantibodies; Carbamates; Citrulline; Humans; Logistic Models; Lysine; Multivariate Analysis; Ornithine; Peptides; Peptides, Cyclic; Prognosis; Randomized Controlled Trials as Topic; Recurrence; Vimentin	2017
Drastic decrease of global l-arginine bioavailability during orthotopic liver transplantation: A matter of ATP deficiency of the graft? Nitric oxide : biology and chemistry, 2018, 02-28, Volume: 73 Topics: Adenosine Triphosphate; Arginine; Biological Availability; Citrulline; Humans; Liver Transplantation; Ornithine	2018