ammonium hydroxide and orotic acid

ammonium hydroxide has been researched along with orotic acid in 144 studies

Research

Studies (144)

Timeframe	Studies, this research(%)	All Research%
pre-1990	110 (76.39)	18.7374
1990's	24 (16.67)	18.2507
2000's	6 (4.17)	29.6817
2010's	1 (0.69)	24.3611
2020's	3 (2.08)	2.80

Authors

Authors	Studies
Bueno, L	1
Alfant, M; Crandall, DE; Knott, CE; Tremblay, GS	1
Bachmann, C; Berüter, J; Colombo, JP; Peheim, E	1
Visek, WJ	5
Ha, YH; Milner, JA	1
Böhles, H; Fekl, W; Harms, D; Heid, H; Sitzmann, FC	1
Corbin, JE; Ha, YH; Milner, JA	1
Hatchwell, LC; Milner, JA	2
Batshaw, ML; Bursilow, SW	1
Hokanson, JT; Idemoto, J; O'Brien, WE; Schafer, IA	1
Milner, JA; Visek, WJ	4
Crandall, DE; Lovatt, CJ; Tremblay, GC	1
Bieth, R; Dreyfus, J; Flori, E; Levy, JM; Lutz, P; Stoll, C	1
Brusilow, S; Mantagos, S; McReynolds, JW; Rosenberg, LE	1
Letarte, J; Quellet, R; Qureshi, IA	1
Thaler, MM	1
Glasgow, AM	1
Brusso, T; Spate, M; Whelan, DT	1
Dhondt, JL; Farriaux, JP	1
Brandt, JT; Fausto, N; Kesner, L	1
Prior, RL; Visek, WJ; Zimber, A	1
Beaudry, MA; Collu, R; Dallairf, L; Ducharme, JR; Leboeuf, G; Letarte, J; Melancon, SB	1
Shiro, Y; Yabuki, S	1
Bachmann, C; Bordugo, A; Burlina, AB; Colombo, JP; Ferrari, V; Wermuth, B; Zacchello, F	1
Lin, CY; Lin, SP; Seashore, MR; Tuchman, M; Vockley, CM; Vockley, J; Wu, TC	1
Nelson, J; Qureshi, IA; Vasudevan, S	1
Gross, KL; Hartman, WJ; Prior, RL; Ronnenberg, A	1
Bardet, J; Deprun, C; Guillois, B; Kamoun, P; Parvy, P; Rabier, D	1
Holzknecht, RA; Tuchman, M	1
Fujiwara, F; Funahashi, S; Hayashi, A; Nakanishi, T; Saiki, K; Shimizu, A	1
Abe, H; Fukushima, H; Kaku, N; Kumashiro, R; Nakao, K; Nishiyori, J; Ohno, T; Tanikawa, K; Yamashita, F; Yoshino, M	1
Bonham, JR; Carpenter, KH; Clarke, A	1
Hjelm, M; Oberholzer, V; Thomas, S; Wilson, J	1
Boedeker, D; Martens, H	1
Daune, G; Gerhart, F; Grauffel, C; Seiler, N	1
Shoemaker, JD; Visek, WJ	2
Azorín, I; Felipo, V; Grisolía, S; Miñana, MD	1
Batshaw, ML; Naylor, EW; Thomas, GH	1
Bachmann, C; Schmidtke, J; Wendel, U; Wilichowski, E	1
Frezal, J; Kamoun, P; Kaplan, J; Munnich, A; Pelet, A; Rabier, D; Saudubray, JM; Toumas, E	1
Chisena, CA; Cysyk, RL; Monks, A	1
de Silva, LV; Hjelm, M; Oberholzer, VG; Rolles, CJ; Seakins, JW	1
Zieve, L	1
Brusilow, SW; Rajantie, J; Simell, O; Sipilä, I; Valle, DL	1
Kato, T; Mizutani, N; Sano, M	1
Motyl, T; Orzechowski, A; Pierzynowski, S	2
Bachmann, C; Colombo, JP; Dionisi Vici, C; Gambarara, M; Sabetta, G	1
Coskun, T; Kneer, J; Mönch, S; Ozalp, I	1
Bachmann, C; Grossschädl, F; Roscher, A; Stöckler, S	1
Girgis, N; Herrin, J; McGravey, V; Shah, BL; Shih, VE	1
Kukulska, W; Motyl, T; Orzechowski, A	1
de Parscau, L; Divry, P; Guibaud, P; Hermier, M; Vianey-Liaud, C	1
Bachmann, C	1
Brusilow, SW; Buffington, CA; Carey, GP; Hargrove, D; Kime, Z; Morris, JG; Rogers, QR	1
Amit, R; Brand, N; Bujanover, Y; Rottem, M; Statter, M; Yatziv, S	1
Hjelm, M; Kay, JD; Oberholzer, VG; Seakins, JW	1
Barth, CA; de Vrese, M	1
Benjamin, LE; Steele, RD	1
Brusilow, SW; Newman, SL; Rowe, PC	1
Milner, JA	1
Goodman, MW; Zieve, L	1
Clow, CL; Mackenzie, S; Scriver, CR; Simell, O	1
Gushiken, T; Saheki, T; Yoshimura, N	1
Saito, S	1
Holtzman, NA	1
Bresnick, E; Lyman, K; Mayfield, ED	1
Kunz, W; Niessing, J; Schnieders, B; Sekeris, CE	1
Hedegaard, J; Le Gal, ML; Le Gal, Y	1
Milner, JA; Visek, WJ; Wakeling, AE	1
Szám, I	1
Hoogenraad, NJ; Kretchmer, N; Levine, RL	1
Cann, HM; Fukanaga, K; Goldstein, AS; Hoogenraad, NJ; Johnson, JD; Sunshine, P; Swierczewski, E	1
Abzug-Horowitz, S; Pinson, A; Russell, A; Statter, M	1
Buist, NR; Hepburn, CA; Kennaway, NG; Ramberg, DA; Strandholm, JJ	1
Arashima, I	1
Freeman, JM; Levy, HL; Lindenbaum, JE; Sunshine, P	1
Mackenzie, S; MacLeod, P; Scriver, CR	1
Dési, I; László, G; Nemesánszky, E; Szelényi, I	1
Desi, I; Farkas, I; Nemesánszky, E; Szelenyi, I	1
Anakura, M; Arashima, S; Matsuda, I; Nambu, H; Takekoshi, Y	1
Alleyne, GA	1
DeHackbeil, KF; Lis, AW; Lis, EW	1
Caroli, J; Goutand, G; Lemonnier, F	1
Hager, SE; Jones, ME	1
Ishihara, H; Wang, SY	1
Larochelle, J; Lemieux, B; Letarte, J; Ouellet, R; Qureshi, IA	1
Barry, DM; Becroft, DM; Simmonds, HA; Webster, DR	1
Cerra, FB; Goodman, MW; Konstantinides, FN; Zieve, L	1
Nagasaki, T	1
Steele, RD	1
Baker, DH; Czarnecki, GL	1
Fico, ME; Milner, JA; Motyl, T	1
Hevia, P; Kari, FW; Ulman, EA; Visek, WJ	1
Cederbaum, SD; Naylor, EW	1
Danks, DM; Grimes, A; Haan, EA; Hoogenraad, NJ	1
Blanding, JH; Tremblay, GC; Wendler, PA	1
Bachmann, C; Colombo, JP	4
Sakane, Y; Sugimoto, T	1
Kubota, K; Murakami, T; Yamashita, F; Yoshida, I; Yoshino, M	1
Letarte, J; Ouellet, R; Qureshi, IA	1
Ambani, LM; Danthi, V; Dhareshwar, SS; Suchak, RH	1
Altman, RP; Glasgow, AM; Schulman, JD; Stolar, CJ	1
Laberge, C; Lelièvre, M; Letarte, J; Ouellet, R; Qureshi, IA	1
Rajantie, J	2
Carter, M; Donnel, GN; Koch, R; McLaren, J; Ng, WG; Oizumi, J; Shaw, KN	1
Batshaw, ML; Brusilow, SW; Jung, AL; Roan, Y; Rosenberg, LA	1
Daune-Anglard, G; Grauffel, C; Knödgen, B; Sarhan, S; Seiler, N	1
Hartman, WJ; Prior, RL; Torre, PM	1
Deshmukh, DR; Ghole, VS; Nelson, J; Qureshi, IA	1
Cyr, D; Giguère, R; Leblanc, D; Mitchell, G; Qureshi, IA	1
Donati, MA; Filippi, L; Resti, M; Zammarchi, E	1
Anegawa, NJ; Batshaw, ML; Gorry, E; Hyman, SL; McLaughlin, BA; Robinson, MB; Smith, IA; Yudkoff, M	1
de Martinville, B; Demmer, LA; Dowton, SB; Kim, JM	1
Ahrens, MJ; Berry, SA; Markowitz, DJ; Plante, RJ; Tuchman, M; Whitley, CB	1
Schaffner, T; Schlumbom, V; Steiner, F; Vella, S; Wermuth, B; Wiesmann, UN; Zurbrügg, R	1
Fukushige, T; Kakinoki, H; Kobayashi, K; Saheki, T	1
Celli, M; Crifò, C; D'Eufemia, P; Finocchiaro, R; Giardini, O; Salerno, C	1
Lukkarinen, M; Mattila, K; Näntö-Salonen, K; Pulkki, K; Simell, O	1
Batshaw, ML; Chirmule, N; Furth, EE; Gao, GP; Haskal, ZJ; Hughes, J; Magosin, S; Nunes, F; Propert, KJ; Raper, SE; Robinson, MB; Simoes, H; Speicher, L; Tazelaar, J; Wilson, JM; Wivel, NA; Yudkoff, M	1
CAROLI, J; HAGEGE, A; LEMONNIER, F; MASSON, S	1
KESNER, L	1
Kim, SW; McPherson, RL; Wu, G	1
Berger, R; Besley, GT; Cleary, MA; de Koning, TJ; Dorland, L; Duran, M; Mandell, R; Olpin, SE; Poll-The, BT; Shih, VE	1
Batshaw, ML; Bell, P; Cabrera-Luque, J; Gao, GP; Hoang, A; McCarter, RJ; Morizono, H; Moscioni, D; Raper, SE; Sanmiguel, J; Stern, A; Wilson, JM; Wu, D	1
Adachi, M; Kanazawa, M; Kobayashi, K; Kubota, M; Kurokawa, K; Murakami, T; Murayama, K; Nagasaka, H; Ogawa, A; Ogawa, E; Takatani, T; Takayanagi, M; Yamamoto, S; Yorifuji, T	1
Bai, C; Chu, Q; Li, B; Liu, M; Qiu, L; Ruan, Y; Wang, Y; Yang, C	1
Enosawa, S; Fukuda, A; Furukawa, S; Ishida, Y; Ogawa, Y; Sugahara, G; Tateno, C; Umezawa, A; Yamasaki, C; Yanagi, A	1
Akcakaya, P; Allegri, G; Bestas, B; Ellis, E; Engberg, S; Firth, M; Forslöw, A; Gramignoli, R; Häberle, J; Hammarstedt, C; Jakobsson, T; Jorns, C; Makris, G; Maresca, M; Rimann, N; Rooyackers, O; Rosen, B; Scherer, T; Srinivasan, RC; Strom, SC; Thöny, B; van Zuydam, NR; Zabulica, M	1
Hall, PL; Wilcox, WR; Wittenauer, AL	1

Reviews

11 review(s) available for ammonium hydroxide and orotic acid

Article	Year
Ammonia metabolism, urea cycle capacity and their biochemical assessment. Nutrition reviews, 1979, Volume: 37, Issue:9 Topics: Amino Acids; Ammonia; Animals; Arginine; Brain; Citric Acid Cycle; Humans; Nutritional Requirements; Ornithine; Orotic Acid; Polyamines; Quaternary Ammonium Compounds; Urea	1979
Metabolic mechanisms in Reye syndrome. End of a Mystery? American journal of diseases of children (1960), 1976, Volume: 130, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Aspirin; Brain Diseases; Carbamoyl-Phosphate Synthase (Ammonia); Child; Drug Synergism; Fatty Liver; Humans; Liver; Mitochondria, Liver; Mitochondrial Swelling; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Reye Syndrome; Virus Diseases	1976
[A case of ornithine transcarbamylase deficiency presenting severe symptoms in adulthood]. Rinsho shinkeigaku = Clinical neurology, 1992, Volume: 32, Issue:7 Topics: Adult; Ammonia; Arginine; Citrulline; Consciousness Disorders; Female; Glycine; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1992
Conditional deficiencies of ornithine or arginine. Journal of the American College of Nutrition, 1986, Volume: 5, Issue:2 Topics: Amino Acids; Amino Acids, Branched-Chain; Ammonia; Animals; Arginine; Citrulline; Female; Glycine; Hepatic Encephalopathy; Humans; Liver Cirrhosis; Lysine; Models, Biological; Ornithine; Orotic Acid; Pregnancy; Protein Deficiency; Urease	1986
Orotic acid, arginine, and hepatotoxicity. Journal of the American College of Nutrition, 1986, Volume: 5, Issue:2 Topics: Adult; Alcoholism; Ammonia; Animals; Arginine; Dietary Fats; Dietary Proteins; Ethanol; Fasting; Ferrets; Food; Humans; Liver; Liver Diseases, Alcoholic; Liver Regeneration; Male; Models, Biological; Orotic Acid; Rats; Reye Syndrome; Urea	1986
Dietary treatment of inborn errors of metabolism. Annual review of medicine, 1970, Volume: 21 Topics: Ammonia; Carbohydrate Metabolism, Inborn Errors; Diet Therapy; Female; Fructose; Galactosemias; Glycogen Storage Disease; Hartnup Disease; Homocystinuria; Humans; Infant; Lactose Intolerance; Malabsorption Syndromes; Malonates; Metabolism, Inborn Errors; Orotic Acid; Phenylketonurias; Porphyrias; Pregnancy; Pyridoxine; Refsum Disease; Sucrose; Thiamine; Uric Acid	1970
[Hyperammoniemia and its treatment]. Orvosi hetilap, 1973, Oct-28, Volume: 114, Issue:43 Topics: Ammonia; Arginine; Aspartic Acid; Humans; Ketoglutaric Acids; Liver; Malates; Metabolism, Inborn Errors; Ornithine; Orotic Acid	1973
A review: biological and clinical aspects of pyrimidine metabolism. Pediatric research, 1974, Volume: 8, Issue:7 Topics: Ammonia; Animals; Cats; Cell Transformation, Neoplastic; Cytidine; Glucose; Humans; Isoproterenol; Lectins; Lipid Metabolism; Liver Regeneration; Lymphocyte Activation; Mice; Orotic Acid; Purine-Pyrimidine Metabolism, Inborn Errors; Pyrimidine Nucleotides; Rats; Salivary Glands; Thymine; Uracil Nucleotides; Urea; Uridine; Uridine Diphosphate Sugars	1974
Ammonia: its effects on biological systems, metabolic hormones, and reproduction. Journal of dairy science, 1984, Volume: 67, Issue:3 Topics: Ammonia; Animals; Cats; Cattle; Chickens; Cricetinae; Dietary Proteins; Dogs; Estrus; Female; Guinea Pigs; Hormones; Horses; Humans; Inactivation, Metabolic; Insulin; Liver; Luteinizing Hormone; Mice; Orotic Acid; Pregnancy; Progesterone; Rabbits; Rats; Reproduction; Sheep; Species Specificity; Urea	1984
An update of concepts of essential amino acids. Annual review of nutrition, 1984, Volume: 4 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Essential; Ammonia; Animals; Arginine; Atrophy; Child; Choroid; Dietary Proteins; Female; Glucose Tolerance Test; Growth; Histidine; Humans; Insulin; Kidney Failure, Chronic; Male; Middle Aged; Nitrogen; Nutritional Requirements; Orotic Acid; Pregnancy; Retinal Diseases; Reye Syndrome; Urea; Uveal Diseases; Wounds and Injuries	1984
Orotic aciduria and species specificity. Nutrition reviews, 1984, Volume: 42, Issue:8 Topics: Ammonia; Animals; Arginine; Cats; Cattle; Dogs; Humans; Lipids; Multienzyme Complexes; Orotate Phosphoribosyltransferase; Orotic Acid; Orotidine-5'-Phosphate Decarboxylase; Rats; Species Specificity; Swine; Urea	1984

Trials

1 trial(s) available for ammonium hydroxide and orotic acid

Article	Year
Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency. European journal of pediatrics, 2006, Volume: 165, Issue:9 Topics: Age of Onset; Amino Acids; Ammonia; Analysis of Variance; Arginine; Biomarkers; Blood Proteins; Body Height; Body Weight; Child, Preschool; Cholesterol, HDL; Cholesterol, LDL; Diet, Protein-Restricted; Growth; Growth Hormone; Humans; Hyperammonemia; Insulin-Like Growth Factor Binding Protein 3; Insulin-Like Growth Factor I; Japan; Male; Nutritional Physiological Phenomena; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Thyrotropin; Time Factors; Treatment Outcome; Triglycerides; Urea	2006

Article

Year

Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency.

European journal of pediatrics, 2006, Volume: 165, Issue:9

Topics: Age of Onset; Amino Acids; Ammonia; Analysis of Variance; Arginine; Biomarkers; Blood Proteins; Body Height; Body Weight; Child, Preschool; Cholesterol, HDL; Cholesterol, LDL; Diet, Protein-Restricted; Growth; Growth Hormone; Humans; Hyperammonemia; Insulin-Like Growth Factor Binding Protein 3; Insulin-Like Growth Factor I; Japan; Male; Nutritional Physiological Phenomena; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Thyrotropin; Time Factors; Treatment Outcome; Triglycerides; Urea

2006

Other Studies

132 other study(ies) available for ammonium hydroxide and orotic acid

Article	Year
Orotic acid and non-protein nitrogen overload in sheep. Annales de recherches veterinaires. Annals of veterinary research, 1979, Volume: 10, Issue:1 Topics: Acetates; Ammonia; Animals; Eating; Female; Gastrointestinal Motility; Hydrogen-Ion Concentration; Nitrogen; Orotic Acid; Rumen; Sheep; Urea	1979
Orotic acid biosynthesis in rat liver: studies on the source of carbamoylphosphate. Archives of biochemistry and biophysics, 1977, Jan-15, Volume: 178, Issue:1 Topics: Ammonia; Ammonium Chloride; Animals; Bicarbonates; Carbamates; Carbamoyl-Phosphate Synthase (Ammonia); Carbamyl Phosphate; Carbon Radioisotopes; Glutamine; Liver; Mitochondria, Liver; Ornithine; Orotic Acid; Phosphotransferases; Putrescine; Rats	1977
Enzymes of ammonia detoxication after portacaval shunt in the rat. I. Carbamylphosphate synthetase and aspartate transcarbamylase. Enzyme, 1977, Volume: 22, Issue:6 Topics: Ammonia; Animals; Aspartate Carbamoyltransferase; Body Weight; Carbamoyl-Phosphate Synthase (Ammonia); DNA; Liver; Male; Organ Size; Orotic Acid; Phosphotransferases; Portacaval Shunt, Surgical; Rats	1977
Urea cycle operation in the arginine deficient rat. Biochemical medicine, 1979, Volume: 22, Issue:2 Topics: Ammonia; Animals; Arginase; Arginine; Argininosuccinate Lyase; Argininosuccinate Synthase; Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Citrulline; Liver; Male; Ornithine; Ornithine Carbamoyltransferase; Orotic Acid; Rats; Urea	1979
Protein load in argininosuccinic aciduria: thoughts on its biochemical implications. Zeitschrift fur Ernahrungswissenschaft, 1978, Volume: 17, Issue:2 Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Arginine; Argininosuccinic Acid; Argininosuccinic Aciduria; Dietary Proteins; Female; Humans; Milk Proteins; Orotic Acid; Urea	1978
Arginine requirements in immature dogs. The Journal of nutrition, 1978, Volume: 108, Issue:2 Topics: Amino Acids; Ammonia; Animals; Arginine; Citrates; Dogs; Hyperglycemia; Nutritional Requirements; Orotic Acid; Sialorrhea; Tremor; Urea	1978
Amino acid induced orotic aciduria. The Journal of nutrition, 1978, Volume: 108, Issue:4 Topics: Amino Acids; Ammonia; Animal Nutritional Physiological Phenomena; Animals; Arginine; Fasting; Male; Ornithine; Orotic Acid; Quaternary Ammonium Compounds; Rats; Urea; Urease	1978
Asymptomatic hyperammonemia in low birthweight infants. Pediatric research, 1978, Volume: 12, Issue:3 Topics: Ammonia; Female; Humans; Infant, Low Birth Weight; Infant, Newborn; Infant, Newborn, Diseases; Infant, Small for Gestational Age; Ketoglutaric Acids; Male; Orotic Acid	1978
Carrier detection in ornithine transcarbamylase deficiency. The Journal of pediatrics, 1978, Volume: 93, Issue:1 Topics: Adolescent; Adult; Ammonia; Child; Child, Preschool; Dietary Proteins; Female; Heterozygote; Humans; Infant, Newborn; Male; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree	1978
Orotic aciduria in the female rat and its relation to dietary arginine. The Journal of nutrition, 1978, Volume: 108, Issue:8 Topics: Aging; Ammonia; Animals; Arginine; Birth Weight; Body Weight; Citrates; Female; Litter Size; Orotic Acid; Pregnancy; Pregnancy Complications; Rats; Species Specificity; Urea	1978
Regulation of pyrimidine biosynthesis by purine and pyrimidine nucleosides in slices of rat tissue. Archives of biochemistry and biophysics, 1978, Volume: 188, Issue:1 Topics: Adenosine; Ammonia; Animals; Bicarbonates; Cytidine; In Vitro Techniques; Liver; Male; Orotic Acid; Pyrimidines; Rats; Ribonucleosides; Spleen; Uridine	1978
[A new family with mutation of the structural gene of human ornithine carbamoyltransferase]. Archives francaises de pediatrie, 1978, Volume: 35, Issue:5 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Genes; Heterozygote; Humans; Infant, Newborn; Male; Mutation; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree	1978
Treatment of complete ornithine transcarbamylase deficiency with nitrogen-free analogues of essential amino acids. The Journal of pediatrics, 1978, Volume: 93, Issue:3 Topics: Amino Acids; Amino Acids, Essential; Ammonia; Child Development; Dietary Proteins; Energy Intake; Growth; Humans; Infant; Infant, Newborn; Male; Nitrogen; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1978
Factors affecting amino acid induced orotic aciduria in rats. The Journal of nutrition, 1978, Volume: 108, Issue:12 Topics: Ammonia; Animals; Dietary Proteins; Digestion; Glycine; Male; Nitrogen; Orotic Acid; Rats; Starvation; Urea	1978
Study of enzyme defect in a case of ornithine transcarbamylase deficiency. Diabete & metabolisme, 1978, Volume: 4, Issue:4 Topics: Ammonia; Child; Female; Humans; Kinetics; Liver; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1978
Potential hazard of therapy for Reye's syndrome with urea cycle intermediates. Pediatrics, 1977, Volume: 60, Issue:4 Topics: Ammonia; Humans; Ornithine; Orotic Acid; Reye Syndrome; Urea	1977
Citrullinemia: phenotypic variations. Pediatrics, 1976, Volume: 57, Issue:6 Topics: Amino Acids; Ammonia; Argininosuccinate Synthase; Blood Urea Nitrogen; Citrulline; Female; Humans; Infant; Orotic Acid; Phenotype	1976
[Trial of detection of female carriers for the ornithine-carbamyltransferase deficiency by the urine assay of crotic acid. Apropos of a family study]. Annales de genetique, 1975, Volume: 18, Issue:3 Topics: Ammonia; Creatinine; Cytoplasm; Female; Genealogy and Heraldry; Genetic Counseling; Heterozygote; Humans; Male; Mitochondria; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Proteins; Purine-Pyrimidine Metabolism, Inborn Errors; Pyrimidines; Sex Chromosomes	1975
Possible interactions between the urea cycle and synthesis of pyrimidines and polyamines in regenerating liver. Cancer research, 1975, Volume: 35, Issue:2 Topics: Adrenalectomy; Ammonia; Animals; Bicarbonates; Carbon Radioisotopes; Carboxy-Lyases; Hepatectomy; Liver; Liver Regeneration; Male; Ornithine; Orotic Acid; Polyamines; Pyrimidines; Rats; Urea; Urease	1975
Citric, orotic, and other organic acids in rats injected with active or inactive urease. The American journal of physiology, 1975, Volume: 228, Issue:3 Topics: Acetates; Ammonia; Animals; Arginine; Blood Glucose; Citrates; Formates; Ketoglutaric Acids; Lactates; Malates; Male; Orotic Acid; Pyruvates; Rats; Succinates; Time Factors; Urea; Urease	1975
Urinary metabolites characteristic of urea-cycle amino acid deficiency. Metabolism: clinical and experimental, 1975, Volume: 24, Issue:5 Topics: Amino Acids; Amino Acids, Essential; Ammonia; Animal Nutritional Physiological Phenomena; Animals; Arginine; Biotransformation; Body Weight; Carbamyl Phosphate; Citrates; Citrulline; Deficiency Diseases; Diet; Male; Nitrogen; Ornithine; Orotic Acid; Pyrimidines; Rats; Urea	1975
[Chronic hyperammonemia with orotic aciduria: evidence of pyrimidine pathway stimulation (author's transl)]. Diabete & metabolisme, 1975, Volume: 1 Topics: Alanine; Amino Acids; Ammonia; Child, Preschool; Dietary Proteins; Erythrocytes; Growth Disorders; Humans; Intellectual Disability; Leukocytes; Ornithine; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotate Phosphoribosyltransferase; Orotic Acid; Orotidine-5'-Phosphate Decarboxylase; Purine-Pyrimidine Metabolism, Inborn Errors; Pyrimidines; Seizures; Syndrome; Urea	1975
Partial N-acetylglutamate synthetase deficiency: a new case with uncontrollable movement disorders. Journal of inherited metabolic disease, 1992, Volume: 15, Issue:3 Topics: Acetyltransferases; Amino-Acid N-Acetyltransferase; Ammonia; Arginine; Benzoates; Benzoic Acid; Brain; Dietary Proteins; Female; Humans; Infant; Magnetic Resonance Imaging; Movement Disorders; Orotic Acid	1992
Nitrogen-stimulated orotic acid synthesis and nucleotide imbalance. Cancer research, 1992, Apr-01, Volume: 52, Issue:7 Suppl Topics: Ammonia; Animals; Carbamyl Phosphate; Cattle; Humans; Liver Neoplasms; Milk; Orotic Acid; Pyrimidines; Rats; Triglycerides; Urea	1992
Normal N-acetylglutamate concentration measured in liver from a new patient with N-acetylglutamate synthetase deficiency: physiologic and biochemical implications. Biochemical medicine and metabolic biology, 1992, Volume: 47, Issue:1 Topics: Acetyltransferases; Amino Acids; Amino-Acid N-Acetyltransferase; Ammonia; Child, Preschool; Crystallization; Glutamates; Humans; Liver; Male; Microscopy, Electron; Mitochondria, Liver; Orotic Acid	1992
[Mechanism of the effect of serine and threonine on ammoniagenesis and biosynthesis of orotate in the mouse]. Clinical and investigative medicine. Medecine clinique et experimentale, 1992, Volume: 15, Issue:2 Topics: Adenine; Ammonia; Animals; Aspartic Acid; Cycloheximide; Male; Mice; Mice, Inbred ICR; Orotic Acid; Phosphonoacetic Acid; Serine; Threonine	1992
Arginine-deficient diets alter plasma and tissue amino acids in young and aged rats. The Journal of nutrition, 1991, Volume: 121, Issue:10 Topics: Aging; Amino Acids; Ammonia; Animals; Arginine; Diet; Liver; Male; Orotic Acid; Rats; Rats, Inbred Strains; Spleen	1991
Ornithine carbamoyltransferase deficiency with subnormal enzyme activity. Journal of inherited metabolic disease, 1991, Volume: 14, Issue:5 Topics: Ammonia; Humans; Infant; Liver; Male; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1991
Heterogeneity of patients with late onset ornithine transcarbamylase deficiency. Clinical and investigative medicine. Medecine clinique et experimentale, 1991, Volume: 14, Issue:4 Topics: Adolescent; Adult; Age Factors; Ammonia; Child; Child, Preschool; Female; Genetic Variation; Glutamine; Heterozygote; Humans; Infant; Intellectual Disability; Liver; Male; Mutation; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Phenotype	1991
Quantitative analysis of urinary pyroglutamic acid in patients with hyperammonemia. Clinica chimica acta; international journal of clinical chemistry, 1991, Mar-29, Volume: 197, Issue:3 Topics: Adult; Aged; Aged, 80 and over; Amino Acid Metabolism, Inborn Errors; Ammonia; Carbamoyl-Phosphate Synthase (Ammonia); Child; Child, Preschool; Female; Gas Chromatography-Mass Spectrometry; Glutamine; Humans; Infant; Infant, Newborn; Male; Middle Aged; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pyrrolidonecarboxylic Acid; Urea	1991
Ornithine transcarbamylase deficiency in male adolescence and adulthood. Enzyme, 1990, Volume: 43, Issue:3 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Dietary Proteins; Female; Genes, Dominant; Humans; Immunoblotting; Liver; Male; Middle Aged; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree	1990
Rett's syndrome and ornithine carbamoyltransferase deficiency. Journal of inherited metabolic disease, 1990, Volume: 13, Issue:3 Topics: Alanine; Ammonia; Female; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Rett Syndrome	1990
The urea cycle in the Rett syndrome. Brain & development, 1990, Volume: 12, Issue:1 Topics: Adult; Ammonia; Child; Child, Preschool; Female; Humans; Middle Aged; Orotic Acid; Rett Syndrome; Urea	1990
Urinary orotic acid excretion in sheep: effects of nitrogen, glucose and arginine. The Journal of nutrition, 1990, Volume: 120, Issue:9 Topics: Abomasum; Ammonia; Animals; Arginine; Aspartate Aminotransferases; Bilirubin; Energy Intake; Feces; Glucose; Male; Nitrogen; Orotic Acid; Rumen; Sheep; Urea	1990
Ornithine aminotransferase activity, liver ornithine concentration and acute ammonia intoxication. Life sciences, 1989, Volume: 45, Issue:11 Topics: Acetates; Administration, Oral; Ammonia; Animals; Arginine; Creatinine; Eflornithine; Female; Injections, Intraperitoneal; Liver; Mice; Ornithine; Ornithine-Oxo-Acid Transaminase; Orotic Acid; Transaminases; Urea	1989
Orotic acid overproduction in experimental cirrhosis of rats. Experimental and molecular pathology, 1989, Volume: 50, Issue:3 Topics: Ammonia; Animals; Carbon Tetrachloride; Liver; Liver Cirrhosis, Experimental; Male; Orotic Acid; Rats; Rats, Inbred Strains; Urea	1989
A simple animal model of hyperammonemia. Hepatology (Baltimore, Md.), 1989, Volume: 10, Issue:3 Topics: Acetates; Ammonia; Animals; Citrulline; Disease Models, Animal; Glutamate Dehydrogenase; Liver; Male; Orotic Acid; Rats; Rats, Inbred Strains; Urea	1989
False positive alanine tolerance test results in heterozygote detection of urea cycle disorders. The Journal of pediatrics, 1989, Volume: 115, Issue:4 Topics: Alanine; Ammonia; Argininosuccinate Synthase; Argininosuccinic Aciduria; Carbamoyl-Phosphate Synthase (Ammonia); False Positive Reactions; Genetic Carrier Screening; Humans; Ligases; Lyases; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Proteins	1989
DNA analysis of ornithine transcarbamylase deficiency. European journal of pediatrics, 1988, Volume: 147, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child, Preschool; DNA; Female; Genetic Carrier Screening; Genetic Markers; Humans; Infant; Infant, Newborn; Male; Nucleic Acid Hybridization; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree; Polymorphism, Genetic; Polymorphism, Restriction Fragment Length	1988
[Genetic counseling in ornithine carbamoyltransferase deficiency]. Annales de biologie clinique, 1988, Volume: 46, Issue:7 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Chromosome Mapping; Female; Genetic Carrier Screening; Genetic Counseling; Genetic Linkage; Humans; Male; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree; Polymorphism, Restriction Fragment Length; Risk Factors	1988
Influence of ammonium ions on hepatic de novo pyrimidine biosynthesis. Archives of biochemistry and biophysics, 1985, Volume: 236, Issue:1 Topics: Ammonia; Ammonium Chloride; Animals; Bicarbonates; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Chromatography, High Pressure Liquid; Glutamine; In Vitro Techniques; Liver; Male; Orotic Acid; Perfusion; Pyrimidines; Rats; Rats, Inbred Strains; Sodium Bicarbonate; Uridine; Uridine Monophosphate; Uridine Triphosphate	1985
Evidence of inherited urea cycle defect in a case of fatal valproate toxicity. British medical journal (Clinical research ed.), 1986, Jan-04, Volume: 292, Issue:6512 Topics: Adult; Ammonia; Child; Child, Preschool; Female; Humans; Male; Metabolism, Inborn Errors; Middle Aged; Orotic Acid; Urea; Valproic Acid	1986
Waste nitrogen excretion via amino acid acylation: benzoate and phenylacetate in lysinuric protein intolerance. Pediatric research, 1986, Volume: 20, Issue:11 Topics: Acylation; Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Benzoates; Benzoic Acid; Child; Child, Preschool; Creatinine; Glutamine; Hippurates; Humans; Lysine; Nitrogen; Orotic Acid; Phenylacetates; Urea	1986
Inhibitory effect of intravenous lysine infusion on urea cycle metabolism. European journal of pediatrics, 1987, Volume: 146, Issue:1 Topics: Adolescent; Amino Acids; Ammonia; Child; Citric Acid Cycle; Citrulline; Depression, Chemical; Female; Humans; Infusions, Intravenous; Lysine; Male; Orotic Acid; Putrescine; Urea	1987
Urinary orotic acid excretion in hyperammonaemic sheep. I. Effect of portal and peripheral hyperammonaemia. Zentralblatt fur Veterinarmedizin. Reihe A, 1987, Volume: 34, Issue:7 Topics: Ammonia; Animals; Female; Orotic Acid; Portal System; Sheep	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
Lethal hyperammonaemic coma due to ornithine transcarbamylase deficiency presenting as brain stem encephalitis in a previously asymptomatic ten-year-old boy. Journal of inherited metabolic disease, 1987, Volume: 10, Issue:3 Topics: Ammonia; Brain Stem; Child; Citrulline; Coma; Diagnosis, Differential; Encephalitis; Humans; Male; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1987
Ornithine transcarbamylase variant in a male patient. Journal of inherited metabolic disease, 1987, Volume: 10, Issue:3 Topics: Amino Acids; Ammonia; Citrulline; Dietary Proteins; Genetic Variation; Humans; Hydrogen-Ion Concentration; Infant; Liver; Male; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1987
Lethal ornithine transcarbamylase deficiency in a female neonate. Journal of inherited metabolic disease, 1987, Volume: 10, Issue:3 Topics: Ammonia; Arginine; Citrulline; Coma; Female; Humans; Infant, Newborn; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Peritoneal Dialysis; Stevens-Johnson Syndrome	1987
Urinary orotic acid excretion in hyperammonaemic sheep. II. Renal clearance of orotic acid. Zentralblatt fur Veterinarmedizin. Reihe A, 1988, Volume: 35, Issue:3 Topics: Ammonia; Animals; Female; Kidney; Orotic Acid; Sheep; Sheep Diseases	1988
[Protein intolerance with lysinuria. Value of orotic aciduria in adjusting treatment with citrulline]. Archives francaises de pediatrie, 1988, Volume: 45, Issue:10 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Amino Acids, Diamino; Ammonia; Biological Transport; Cell Membrane; Citrulline; Consanguinity; Dietary Proteins; Humans; Lysine; Male; Orotic Acid; Osteoporosis	1988
The relationship between urea and pyrimidine de novo synthesis in ruminant liver. Quarterly journal of experimental physiology (Cambridge, England), 1988, Volume: 73, Issue:1 Topics: Amino Acids; Ammonia; Animals; Biological Transport; Female; Liver; Orotic Acid; Pyrimidines; Sheep; Urea	1988
Diagnosis of urea cycle disorders. Enzyme, 1987, Volume: 38, Issue:1-4 Topics: Algorithms; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Carboxylic Acids; Carnitine; Humans; Orotic Acid	1987
An arginine-deficient diet in humans does not evoke hyperammonemia or orotic aciduria. The Journal of nutrition, 1987, Volume: 117, Issue:10 Topics: Adult; Amino Acids; Ammonia; Arginine; Diet; Female; Humans; Male; Orotic Acid	1987
Clinical and laboratory study in 22 patients with inherited hyperammonemic syndromes. Israel journal of medical sciences, 1986, Volume: 22, Issue:11 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Coma; Female; Humans; Male; Orotic Acid; Pedigree; Syndrome	1986
Effect of partial ornithine carbamoyltransferase deficiency on urea synthesis and related biochemical events. Clinical science (London, England : 1979), 1987, Volume: 72, Issue:2 Topics: Alanine; Ammonia; Child; Female; Humans; Kinetics; Male; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Urea	1987
Arginine and disease states. The Journal of nutrition, 1985, Volume: 115, Issue:4 Topics: Ammonia; Animals; Arginine; Blood Glucose; Humans; Insulin; Lipid Metabolism; Liver Diseases; Male; Nutritional Requirements; Orotic Acid; Retinal Degeneration; Reye Syndrome; Spermatogenesis; Urea	1985
Influence of lysine on urea cycle activity and orotate formation in the isolated perfused rat liver. Biological chemistry Hoppe-Seyler, 1985, Volume: 366, Issue:5 Topics: Ammonia; Animals; In Vitro Techniques; Kinetics; Liver; Lysine; Male; Ornithine; Orotic Acid; Perfusion; Rats; Rats, Inbred Strains; Urea	1985
The effect of dietary protein on nitrogen and sulfur metabolism in portacaval-shunted rats. The Journal of nutrition, 1986, Volume: 116, Issue:1 Topics: Amino Acids, Sulfur; Ammonia; Animals; Body Weight; Dietary Proteins; Hepatic Encephalopathy; Liver; Male; Nitrogen; Orotic Acid; Portacaval Shunt, Surgical; Rats; Rats, Inbred Strains; Sulfur; Taurine	1986
Natural history of symptomatic partial ornithine transcarbamylase deficiency. The New England journal of medicine, 1986, Feb-27, Volume: 314, Issue:9 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Ataxia; Child, Preschool; Coma; Diagnosis, Differential; Dietary Proteins; Female; Food Preferences; Growth Disorders; Humans; Infant; Infant, Newborn; Irritable Mood; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Phenotype; Seizures; Sleep Stages; Vomiting	1986
Metabolic aberrations associated with arginine deficiency. The Journal of nutrition, 1985, Volume: 115, Issue:4 Topics: Ammonia; Animals; Arginine; Citrates; Citric Acid; Diet; Humans; Lipid Metabolism; Liver; Orotic Acid; Purines; Pyrimidines; Urea	1985
Carbamoyl-phosphate synthetase I activity and ureagenesis in regenerating liver of the normal rat. The American journal of physiology, 1985, Volume: 248, Issue:5 Pt 1 Topics: Ammonia; Animals; Carbamoyl-Phosphate Synthase (Ammonia); Ligases; Liver; Liver Regeneration; Male; Orotic Acid; Rats; Rats, Inbred Strains; Urea	1985
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
Transient hyperammonemia during aging in ornithine transcarbamylase-deficient, sparse-fur mice. Biochemistry international, 1985, Volume: 11, Issue:5 Topics: Aging; Ammonia; Animals; Body Weight; Disease Models, Animal; Hair; Liver; Mice; Mice, Mutant Strains; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Urea	1985
[Growth hormone]. Nihon Naibunpi Gakkai zasshi, 1967, Nov-20, Volume: 43, Issue:8 Topics: Alkaline Phosphatase; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Animals; Carbon Isotopes; Dactinomycin; Diaphragm; Dwarfism, Pituitary; Growth Hormone; Humans; Hydroxyproline; In Vitro Techniques; Leucine; Leucyl Aminopeptidase; Liver; Male; Nitrogen; Orotic Acid; Protein Biosynthesis; Puromycin; Pyruvates; Radioisotopes; Rats; RNA	1967
Incorporation of bicarbonate-14 C into pyrimidines and into ribonucleic acid of the Novikoff ascites tumor cell. Cancer research, 1967, Volume: 27, Issue:3 Topics: Ammonia; Animals; Aspartic Acid; Bicarbonates; Carcinoma, Hepatocellular; Chromatography, Ion Exchange; Diazooxonorleucine; Glutamine; In Vitro Techniques; Liver Neoplasms; Neoplasms, Experimental; Orotic Acid; Pyrimidines; Rats; RNA, Neoplasm; Uracil Nucleotides	1967
Characterization of rapidly labelled rat liver ribonucleic acid showing high affinity for columns of methylated albumin on kieselguhr. The Biochemical journal, 1970, Volume: 116, Issue:4 Topics: Albumins; Ammonia; Animals; Centrifugation, Density Gradient; Chromatography; Liver; Male; Methylation; Mice; Molecular Weight; Orotic Acid; Rats; RNA; Saccharomyces; Silicon Dioxide; Tritium	1970
[Regulation of the synthesis of ribosylamine-5-phosphate by the pyrimidines in Escherichia coli B]. Comptes rendus hebdomadaires des seances de l'Academie des sciences. Serie D: Sciences naturelles, 1971, Jan-18, Volume: 272, Issue:3 Topics: Adenine Nucleotides; Ammonia; Cytosine Nucleotides; Diphosphates; Escherichia coli; Glutamine; Nucleotides; Orotic Acid; Purines; Pyrimidines; Ribose; Uracil Nucleotides	1971
Effect of arginine deficiency on growth and intermediary metabolism in rats. The Journal of nutrition, 1974, Volume: 104, Issue:12 Topics: Amino Acids; Ammonia; Animal Nutritional Physiological Phenomena; Animals; Arginine; Body Weight; Citrates; Creatinine; Deficiency Diseases; Diet; Male; Nitrogen; Orotic Acid; Rats; Time Factors; Urea; Urine	1974
Orotate, citrate, and urea excretion in rats fed various levels of arginine. Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1974, Volume: 147, Issue:3 Topics: Ammonia; Ammonium Chloride; Animals; Arginine; Citrates; Diet; Male; Orotic Acid; Rats; Sodium Chloride; Urea	1974
Orotic aciduria and arginine deficiency. Nature, 1973, Sep-28, Volume: 245, Issue:5422 Topics: Ammonia; Animals; Arginine; Deficiency Diseases; Orotic Acid; Rats; Urea	1973
Metabolic and genetic studies of a family with ornithine transcarbamylase deficiency. Pediatric research, 1974, Volume: 8, Issue:1 Topics: Ammonia; Ammonium Chloride; Brain; Citrates; Dietary Proteins; Estradiol; Glucose; Heterozygote; Humans; Infant, Newborn; Intestines; Liver; Male; Metabolism, Inborn Errors; Ornithine Carbamoyltransferase; Orotic Acid; Pedigree; Peritoneal Dialysis; Phenobarbital; Proline; Seizures; Sex Factors	1974
Abnormal orotic acid metabolism associated with acute hyperammonaemia in the rat. Biochemical medicine, 1974, Volume: 9, Issue:1 Topics: Acetates; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Carbon Radioisotopes; Carboxy-Lyases; Chromatography, Ion Exchange; Chromatography, Paper; Disease Models, Animal; Electrophoresis, Paper; Glutamates; Glutamine; Hydrogen-Ion Concentration; Liver; Male; Mass Spectrometry; Orotic Acid; Pentosyltransferases; Purine Nucleotides; Quaternary Ammonium Compounds; Rats; Time Factors; Uracil Nucleotides	1974
Citrullinemia: investigation and treatment over a four-year period. The Journal of pediatrics, 1974, Volume: 85, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Arginine; Child, Preschool; Citrulline; Dietary Proteins; Dipeptides; Female; Fibroblasts; Follow-Up Studies; Guanidines; Humans; Infant; Infant, Newborn; Orotic Acid; Peptide Synthases; Urea; Uric Acid	1974
[Hyperammonemia--congenital abnormality of the urea cycle]. Saishin igaku. Modern medicine, 1972, Volume: 27, Issue:4 Topics: Amino Acids; Ammonia; Animals; Arginase; Carbamates; DNA; Female; Humans; Infant; Kinetics; Ligases; Liver; Male; Metabolism, Inborn Errors; Ornithine Carbamoyltransferase; Orotic Acid; Phosphotransferases; Rats; RNA; Urea	1972
Hyperammonemia due to a defect in hepatic ornithine transcarbamylase. Pediatrics, 1972, Volume: 50, Issue:1 Topics: Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Central Nervous System Diseases; Child; Child Behavior Disorders; Citrates; Coma; Diet Therapy; Dietary Proteins; Female; Gastrointestinal Diseases; Guanidines; Humans; Liver; Male; Ornithine; Ornithine Carbamoyltransferase; Orotic Acid; Succinates; Urea	1972
Partial ornithine carbamyl transferase deficiency: an inborn error of the urea cycle presenting as orotic aciduria in a male infant. Canadian Medical Association journal, 1972, Sep-09, Volume: 107, Issue:5 Topics: Ammonia; Chromatography, Gas; Coma; Humans; Infant; Infusions, Parenteral; Liver; Male; Metabolism, Inborn Errors; Ornithine Carbamoyltransferase; Orotic Acid; Spectrophotometry, Ultraviolet; Urea; Vomiting	1972
[Effect of magnesium orotate glycinate on experimental ammonia poisoning]. Arzneimittel-Forschung, 1971, Volume: 21, Issue:6 Topics: Ammonia; Animals; Carbon Tetrachloride Poisoning; Female; Glycine; Liver; Magnesium; Male; Orotic Acid; Poisoning; Rats	1971
[Effect of magnesium orotate glycinate on the brain electric activity after experimental ammonia poisoning]. Arzneimittel-Forschung, 1971, Volume: 21, Issue:6 Topics: Ammonia; Animals; Brain Diseases; Chemical and Drug Induced Liver Injury; Electroencephalography; Female; Glycine; Magnesium; Orotic Acid; Poisoning; Rats	1971
Hyperammonemia due to a mutant enzyme of ornithine transcarbamylase. Pediatrics, 1971, Volume: 48, Issue:4 Topics: Amino Acids; Ammonia; Animals; Autopsy; Clinical Enzyme Tests; Culture Techniques; Female; Humans; Infant; Liver; Metabolism, Inborn Errors; Mutation; Ornithine; Ornithine Carbamoyltransferase; Orotic Acid; Phosphotransferases; Rats; Threonine; Urea	1971
Renal metabolic response to acid-base changes. II. The early effects of metabolic acidosis on renal metabolism in the rat. The Journal of clinical investigation, 1970, Volume: 49, Issue:5 Topics: Acidosis; Ammonia; Ammonium Chloride; Animals; Aspartic Acid; Carbohydrates; Gluconeogenesis; Ketoglutaric Acids; Kidney; Lactates; Malates; Orotic Acid; Pyruvate Kinase; Pyruvates; Rats; RNA; Tritium	1970
Ultraviolet-absorbing components of urine from mentally retarded children. 3. Clinical chemistry, 1970, Volume: 16, Issue:8 Topics: Adolescent; Adult; Ammonia; Autistic Disorder; Central Nervous System Diseases; Child; Child, Preschool; Chromatography, Ion Exchange; Chromatography, Paper; Citrulline; Creatinine; Dialysis; Humans; Hypnotics and Sedatives; Intellectual Disability; Male; Nephrectomy; Orotic Acid; RNA, Messenger; Sleep; Tranquilizing Agents; Ultraviolet Rays	1970
[Action of lysine on the hyperammoniema of the cirrhotic patient]. La Presse medicale, 1971, May-08, Volume: 7, Issue:23 Topics: Amino Acids; Ammonia; Humans; Injections, Intravenous; Liver Cirrhosis; Lysine; Male; Middle Aged; Orotic Acid; Perfusion; Time Factors	1971
Initial steps in pyrimidine synthesis in Ehrlich ascites carcinoma in vitro. 1. Factors affecting the incorporation of 14C-bicarbonate into carbon 2 of the uracil ring of the acid-soluble nucleotides of intact cells. The Journal of biological chemistry, 1965, Volume: 240, Issue:12 Topics: Ammonia; Animals; Azaserine; Bicarbonates; Carbamates; Carcinoma, Ehrlich Tumor; Glucose; Glutamine; In Vitro Techniques; Orotic Acid; Phosphates; Serine; Uracil; Uracil Nucleotides	1965
Photochemistry of 5-bromouracil in aqueous solution. Biochemistry, 1966, Volume: 5, Issue:7 Topics: Allantoin; Ammonia; Barbiturates; Chemical Phenomena; Chemistry; Orotic Acid; Oxalates; Radiation Effects; Solutions; Ultraviolet Rays; Uracil; Urea	1966
A new French-Canadian family affected by hyperargininaemia. Journal of inherited metabolic disease, 1983, Volume: 6, Issue:4 Topics: Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Child, Preschool; Erythrocytes; Female; Humans; Hyperargininemia; Infant, Newborn; Male; Orotic Acid; Proteins	1983
Failure of protein loading tests to identify heterozygosity for ornithine carbamoyltransferase deficiency. Journal of inherited metabolic disease, 1984, Volume: 7, Issue:4 Topics: Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Dietary Proteins; Female; Genetic Carrier Screening; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1984
Mechanism of arginine protection against ammonia intoxication in the rat. The American journal of physiology, 1984, Volume: 247, Issue:3 Pt 1 Topics: Acetyltransferases; Amino-Acid N-Acetyltransferase; Ammonia; Animals; Arginine; Carbamoyl-Phosphate Synthase (Ammonia); Citrulline; Male; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase; Orotic Acid; Rats; Rats, Inbred Strains; Urea	1984
[Ammonia metabolism during perinatal period--ontogenesis of enzymes in pyrimidine biosynthesis and urea cycle system]. Nihon Sanka Fujinka Gakkai zasshi, 1984, Volume: 36, Issue:12 Topics: Aging; Ammonia; Animals; Animals, Newborn; Aspartate Carbamoyltransferase; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Female; Fetus; Liver; Orotic Acid; Pregnancy; Pyrimidines; Rats; Rats, Inbred Strains; Urea	1984
Hyperammonemia and orotic aciduria in portacaval-shunted rats. The Journal of nutrition, 1984, Volume: 114, Issue:1 Topics: Ammonia; Ammonium Chloride; Animals; Arginine; Benzoates; Benzoic Acid; Liver; Liver Diseases; Male; Nitrogen; Organ Size; Orotic Acid; Portacaval Shunt, Surgical; Rats; Rats, Inbred Strains; Spleen; Urea	1984
Urea cycle function in the dog with emphasis on the role of arginine. The Journal of nutrition, 1984, Volume: 114, Issue:3 Topics: Age Factors; Ammonia; Animals; Arginine; Blood Glucose; Citrulline; Dogs; Ornithine; Orotic Acid; Urea; Vomiting	1984
Species comparison of the influence of ammonia on orotic acid and urea biosynthesis in liver. The Journal of nutrition, 1984, Volume: 114, Issue:3 Topics: Ammonia; Animals; Arginine; Cattle; Female; Liver; Male; Mice; Mice, Inbred ICR; Orotic Acid; Pyrimidines; Rats; Rats, Inbred Strains; Sheep; Species Specificity; Swine; Urea; Valine	1984
Orotic aciduria caused by feeding excess lysine to growing rats. The Journal of nutrition, 1981, Volume: 111, Issue:10 Topics: Ammonia; Animals; Arginine; Body Weight; Creatinine; Eating; Lysine; Male; Orotic Acid; Rats; Rats, Inbred Strains; Urea	1981
Urinary pyrimidine excretion in arginase deficiency. Journal of inherited metabolic disease, 1981, Volume: 4, Issue:4 Topics: Adolescent; Ammonia; Arginine; Child; Child, Preschool; Chromatography, High Pressure Liquid; Humans; Hyperargininemia; Orotic Acid; Pseudouridine; Pyrimidines; Reference Values; Uracil; Uridine	1981
Carrier detection in ornithine transcarbamylase deficiency. Journal of inherited metabolic disease, 1982, Volume: 5, Issue:1 Topics: Adolescent; Adult; Aging; Ammonia; Child; Duodenum; Female; Genetic Carrier Screening; Humans; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1982
Interaction between the urea cycle and the orotate pathway: studies with isolated hepatocytes. Archives of biochemistry and biophysics, 1983, Jul-01, Volume: 224, Issue:1 Topics: Ammonia; Animals; Carbamoyl-Phosphate Synthase (Ammonia); Catalysis; In Vitro Techniques; Ligases; Liver; Male; Ornithine; Orotic Acid; Pyrimidines; Rats; Rats, Inbred Strains; Urea	1983
[Hyperammonemia: a suggestion for diagnostic and therapeutic procedures]. Padiatrie und Padologie, 1982, Volume: 17, Issue:2 Topics: Amino Acids; Ammonia; Benzoates; Child; Child, Preschool; Diagnosis, Differential; Dietary Proteins; Humans; Infant; Infant, Newborn; Lactates; Liver; Orotic Acid	1982
[Changes of urinary excretion of orotic acid in a patient with ornithine carbamyl transferase deficiency]. Rinsho byori. The Japanese journal of clinical pathology, 1982, Volume: 30, Issue:5 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child, Preschool; Female; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1982
Argininemia: report of a new case and mechanisms of orotic aciduria and hyperammonemia. Advances in experimental medicine and biology, 1982, Volume: 153 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Child, Preschool; Female; Humans; Hyperargininemia; Ornithine; Orotic Acid; Purine-Pyrimidine Metabolism, Inborn Errors; Urea	1982
Spontaneous animal models of ornithine transcarbamylase deficiency: studies on serum and urinary nitrogenous metabolites. Advances in experimental medicine and biology, 1982, Volume: 153 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic Acid; Diet; Female; Glutamine; Heterozygote; Liver; Male; Mice; Mice, Mutant Strains; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1982
Orotic acid in urine and hyperammonemia. Advances in experimental medicine and biology, 1982, Volume: 153 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Anion Exchange Resins; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Purine-Pyrimidine Metabolism, Inborn Errors	1982
Inborn errors of metabolism in acutely sick children. The Indian journal of medical research, 1982, Volume: 76 Topics: Acids; Amino Acids; Ammonia; Carbohydrates; Child; Child, Preschool; Chromatography, Thin Layer; Female; Humans; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Orotic Acid	1982
Ammonia metabolism in dogs with portacaval shunts. Journal of pediatric surgery, 1982, Volume: 17, Issue:5 Topics: Ammonia; Animals; Arginine; Body Weight; Citrulline; Dogs; Female; Liver; Nitrogen; Organ Size; Orotic Acid; Portacaval Shunt, Surgical; Urea	1982
Ammonia metabolism in a family affected by hyperargininemia. Diabete & metabolisme, 1981, Volume: 7, Issue:1 Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Arginase; Arginine; Blood Urea Nitrogen; Erythrocytes; Female; Humans; Orotic Acid; Urea	1981
Orotic aciduria in lysinuric protein intolerance: dependence on the urea cycle intermediates. Pediatric research, 1981, Volume: 15, Issue:2 Topics: Adolescent; Adult; Alanine; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Carbamyl Phosphate; Child; Child, Preschool; Female; Heterozygote; Homozygote; Humans; Infant; Male; Orotic Acid; Proteins; Rats; Urea	1981
Carrier detection of urea cycle disorders. Pediatrics, 1981, Volume: 68, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Argininosuccinate Synthase; Citrulline; Female; Genetic Carrier Screening; Humans; Infant, Newborn; Male; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree; Pregnancy; Prenatal Diagnosis; Urea	1981
[Urinary orotic acid excretion - a sensitive indicator of ammonia detoxication]. Duodecim; laaketieteellinen aikakauskirja, 1981, Volume: 97, Issue:22 Topics: Ammonia; Humans; Metabolism, Inborn Errors; Orotic Acid	1981
Cerebral dysfunction in asymptomatic carriers of ornithine transcarbamylase deficiency. The New England journal of medicine, 1980, Feb-28, Volume: 302, Issue:9 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Brain; Dietary Proteins; Female; Genetic Carrier Screening; Heterozygote; Humans; Infant; Infant, Newborn; Male; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree; Wechsler Scales	1980
Determination of orotic acid in children's urine. Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie, 1980, Volume: 18, Issue:5 Topics: Adolescent; Ammonia; Child; Child, Preschool; Chromatography, Ion Exchange; Diagnosis, Differential; Humans; Infant; Infant, Newborn; Orotic Acid	1980
Diagnostic value of orotic acid excretion in heritable disorders of the urea cycle and in hyperammonemia due to organic acidurias. European journal of pediatrics, 1980, Volume: 134, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Child; Child, Preschool; Citrulline; Female; Humans; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Middle Aged; Orotic Acid	1980
Decreased hyperammonaemia and orotic aciduria due to inactivation of ornithine aminotransferase in mice with a hereditary abnormal ornithine carbamoyltransferase. Journal of inherited metabolic disease, 1994, Volume: 17, Issue:6 Topics: Amino Acids; Ammonia; Animals; Behavior, Animal; Female; Male; Metabolism, Inborn Errors; Mice; Mice, Mutant Strains; Mutation; Ornithine; Ornithine Carbamoyltransferase; Ornithine-Oxo-Acid Transaminase; Orotic Acid	1994
Dietary citrulline but not ornithine counteracts dietary arginine deficiency in rats by increasing splanchnic release of citrulline. The Journal of nutrition, 1994, Volume: 124, Issue:10 Topics: Alanine; Amino Acids; Ammonia; Animals; Arginine; Blood Flow Velocity; Body Weight; Citrulline; Creatinine; Diet; Food; Glutamic Acid; Glutamine; Male; Ornithine; Orotic Acid; Proline; Rats; Rats, Sprague-Dawley; Splanchnic Circulation; Urea	1994
Regulation of orotic acid biosynthesis and excretion induced by oral glutamine administration in mice. Biochemical medicine and metabolic biology, 1993, Volume: 49, Issue:3 Topics: Administration, Oral; Ammonia; Animals; Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Carbamyl Phosphate; Cycloheximide; Glutamine; Isoxazoles; Male; Mice; Mice, Inbred ICR; Mitochondria, Liver; Ornithine Decarboxylase; Ornithine-Oxo-Acid Transaminase; Orotic Acid	1993
Breeding experiments to combine the X-linked sparse-fur (spf) mutation with the autosomal recessive BALB/cByJ strain: testing the biochemical phenotype of double-mutant mice as a model for ammonia: fatty acyl CoA synergism. Biochemical and biophysical research communications, 1993, Mar-15, Volume: 191, Issue:2 Topics: Acyl Coenzyme A; Acyl-CoA Dehydrogenases; Ammonia; Animals; Butyryl-CoA Dehydrogenase; Crosses, Genetic; Female; Genes, Recessive; Genetic Linkage; Glycine; Hair; Male; Malonates; Mice; Mice, Inbred BALB C; Mice, Mutant Strains; Mutation; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Phenotype; Succinates; X Chromosome	1993
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
The sparse fur mouse as a model for gene therapy in ornithine carbamoyltransferase deficiency. Gene therapy, 1995, Volume: 2, Issue:10 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Animals; Avoidance Learning; Crosses, Genetic; Disease Models, Animal; Female; Fertility; Genetic Therapy; Hair; Humans; Male; Mice; Mice, Mutant Strains; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pregnancy	1995
A novel missense mutation in the exon containing the putative ornithine-binding domain of the OTC enzyme in a female. Human mutation, 1996, Volume: 7, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Binding Sites; Child, Preschool; Codon, Terminator; DNA Probes; Exons; Female; Humans; Mutation; Ornithine; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	1996
Clinical and biochemical heterogeneity in females of a large pedigree with ornithine transcarbamylase deficiency due to the R141Q mutation. American journal of medical genetics, 1996, Dec-18, Volume: 66, Issue:3 Topics: Allopurinol; Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Female; Glutamine; Heterozygote; Humans; Infant, Newborn; Male; Mutation; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pedigree	1996
Mutation of ornithine transcarbamylase (H136R) in a girl with severe intermittent orotic aciduria but normal enzyme activity. Journal of inherited metabolic disease, 1997, Volume: 20, Issue:4 Topics: Ammonia; Child; Female; Humans; Infant; Kinetics; Liver; Microscopy, Electron; Mitochondria, Liver; Mutation; Ornithine Carbamoyltransferase; Orotic Acid; Polymerase Chain Reaction; Proteins	1997
No increase of ornithine concentration in the liver of ornithine transcarbamylase-deficient spf-ash mice following intraperitoneal injection of ammonium chloride. Contributions to nephrology, 1997, Volume: 121 Topics: Ammonia; Ammonium Chloride; Animals; Arginine; Citrulline; Liver; Mice; Mice, Inbred Strains; Mice, Transgenic; Ornithine; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Peptones	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
Effect of lysine infusion on urea cycle in lysinuric protein intolerance. Metabolism: clinical and experimental, 2000, Volume: 49, Issue:5 Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Amino Acids; Ammonia; Carrier Proteins; Female; Humans; Lysine; Male; Membrane Proteins; Middle Aged; Mutation; Orotic Acid; Urea	2000
A pilot study of in vivo liver-directed gene transfer with an adenoviral vector in partial ornithine transcarbamylase deficiency. Human gene therapy, 2002, Jan-01, Volume: 13, Issue:1 Topics: Adenoviruses, Human; Adolescent; Adult; Aged; Amino Acids; Ammonia; Female; Gene Transfer Techniques; Genetic Therapy; Genetic Vectors; Heterozygote; Humans; In Situ Hybridization; Liver; Male; Middle Aged; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Pilot Projects; Safety	2002
[A NEW TREATMENT OF EXCESS BLOOD AMMONIA IN CIRRHOTIC PATIENTS: LYSINE OROTATE]. Revue medico-chirurgicale des maladies du foie, 1964, Volume: 39 Topics: Ammonia; Blood Chemical Analysis; Drug Therapy; Humans; Liver Cirrhosis; Lysine; Orotic Acid	1964
THE EFFECT OF AMMONIA ADMINISTRATION ON OROTIC ACID EXCRETION IN RATS. The Journal of biological chemistry, 1965, Volume: 240 Topics: Ammonia; Ammonium Chloride; Antimetabolites; Arginine; Chromatography; Citrulline; Diet; Glutamine; Ornithine; Orotic Acid; Pharmacology; Rats; Research; Succinates; Urine	1965
Dietary arginine supplementation enhances the growth of milk-fed young pigs. The Journal of nutrition, 2004, Volume: 134, Issue:3 Topics: Amino Acids; Amino Acids, Essential; Ammonia; Animals; Animals, Newborn; Arginine; Blood Glucose; Body Weight; Dietary Supplements; Growth Hormone; Insulin; Milk; Orotic Acid; Swine; Urea	2004
Ornithine aminotransferase deficiency: diagnostic difficulties in neonatal presentation. Journal of inherited metabolic disease, 2005, Volume: 28, Issue:5 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Diagnosis, Differential; Female; Fibroblasts; Glutamine; Humans; Hyperammonemia; Infant, Newborn; Male; Mutation; Neonatal Screening; Ornithine; Ornithine-Oxo-Acid Transaminase; Orotic Acid	2005
Long-term correction of ammonia metabolism and prolonged survival in ornithine transcarbamylase-deficient mice following liver-directed treatment with adeno-associated viral vectors. Molecular therapy : the journal of the American Society of Gene Therapy, 2006, Volume: 14, Issue:1 Topics: Ammonia; Animals; Behavior, Animal; Dependovirus; Female; Genetic Therapy; Genetic Vectors; Liver; Male; Mice; Mice, Inbred C3H; Mice, Inbred C57BL; Mice, Knockout; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Survival Analysis; Time Factors	2006
Coordinative metabolism of glutamine carbon and nitrogen in proliferating cancer cells under hypoxia. Nature communications, 2019, 01-14, Volume: 10, Issue:1 Topics: Acetyl Coenzyme A; Ammonia; Animals; Carbon; Cell Hypoxia; Cell Line, Tumor; Cell Survival; Female; Glucose; Glutamine; HEK293 Cells; Humans; Lactic Acid; Lipogenesis; Metabolic Networks and Pathways; Metabolome; Metabolomics; Mice; Mice, Nude; Neoplasms; Nitrogen; Nucleotides; Orotic Acid; Tumor Microenvironment; Xenograft Model Antitumor Assays	2019
Humanized liver mouse model with transplanted human hepatocytes from patients with ornithine transcarbamylase deficiency. Journal of inherited metabolic disease, 2021, Volume: 44, Issue:3 Topics: Ammonia; Animals; Child, Preschool; Disease Models, Animal; Female; Gene Expression Regulation; Hepatocytes; Humans; Infant; Male; Mice; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid	2021
Correction of a urea cycle defect after ex vivo gene editing of human hepatocytes. Molecular therapy : the journal of the American Society of Gene Therapy, 2021, 05-05, Volume: 29, Issue:5 Topics: Adult; Aged; Ammonia; Animals; Cells, Cultured; Child; Disease Models, Animal; Female; Gene Editing; Gene Expression Regulation; Hepatocytes; Humans; Introns; Male; Mice; Mutation; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; RNA Splicing	2021
Proximal urea cycle defects are challenging to detect with newborn screening: Results of a prospective pilot study using post-analytical tools. American journal of medical genetics. Part C, Seminars in medical genetics, 2022, Volume: 190, Issue:2 Topics: Amino Acids; Ammonia; Glutamine; Humans; Infant, Newborn; Lysine; Neonatal Screening; Orotic Acid; Pilot Projects; Prospective Studies; Urea	2022