tryptophan and BH4 Deficiency

tryptophan has been researched along with BH4 Deficiency in 117 studies

Research

Studies (117)

TimeframeStudies, this research(%)All Research%
pre-1990100 (85.47)18.7374
1990's5 (4.27)18.2507
2000's6 (5.13)29.6817
2010's4 (3.42)24.3611
2020's2 (1.71)2.80

Authors

AuthorsStudies
Barone, R; Bensi, G; Biasucci, G; Bonfanti, C; Borghi, E; Burlina, A; Carbone, MT; Cazzorla, C; Dionisi Vici, C; Montanari, C; Parolisi, S; Tosi, M; Verduci, E; Zuvadelli, J1
Besson, G; Boulet, L; Corne, C; Faure, P; Maillot, F; Van Noolen, L1
Levy, HL; Murali, SG; Nair, N; Ney, DM; Rohr, F; Sawin, EA; Stroup, BM1
Besson, G; Boulet, L; Corne, C; Ducros, V; Faure, P1
Azen, C; Moseley, K; Yano, S1
Anikster, Y; Ben-Zeev, B; Benoist, JF; Berutti, R; Blau, N; Bruggmann, R; Cremer, K; Dorboz, I; Engels, H; Gahl, WA; Gemperle-Britschgi, C; Guarani, V; Haack, TB; Harper, JW; Heimer, G; Hoffmann, GF; Huttlin, EL; Imbard, A; Keller, I; Landau, Y; Malicdan, MCV; Marek-Yagel, D; Martinez, A; Mayatepek, E; Meili, D; Meissner, T; Meitinger, T; Mullikin, JC; Opladen, T; Paulo, JA; Pode-Shakked, B; Prokisch, H; Schiff, M; Schwartz, G; Shen, N; Strom, TM; Thöny, B; Trefz, FK; Vilboux, T; Ziv-Strasser, L1
Beattie, D; Bertorelle, G; Byck, S; Cali, F; Croke, DT; Desviat, LR; Dunican, D; Graham, C; Guldberg, P; Hrabincova, E; Jurkowska, M; Knappskog, P; Kozak, L; Kucinskas, V; Livshits, L; Mayne, PD; Naughten, E; Nechyporenko, M; O'Neill, C; Perez, B; Romano, V; Scriver, CR; Tighe, O; Treacy, E; Tyfield, L; Ugarte, M; Zekanowski, C; Zschocke, J1
Koch, R; Moats, RA; Moseley, KD; Nelson, M; Yano, S1
BAIRD, H; BALDRIDGE, RC; BOROFSKY, L; BULLOCK, D; REICHLE, F1
BESSMAN, SP; TADA, K1
SCHULTZE-JENA, BS1
CAREDDU, P; SCHWARZ-TIENE, E1
BRIET, B; CHRISTIAENS, L; GAUDIER, B1
BOGGS, DE; KAPPY, M; MCLAY, D; WAISMAN, HA1
HANSEN, S; HESTRIN, M; PERRY, TL; TISCHLER, B1
KNAPP, A1
FISCHL, J; RABIAH, S1
MURTAGH, JJ; RAPPALLINI, C1
BESSMAN, SP1
APOLLONIO, T; CAREDDU, P; GIOVANNINI, M; TENCONI, L1
GELLER, E; SLATER, GG; YUWILER, A1
GANDULLIA, E; PESCETTO, T; SEGNI, G1
BALDRIDGE, RC; DOBBS, J; REICHLE, FA; TROMPETTER, M1
Embury, JE; Laipis, PJ; Reep, RR1
Artuch, R; Campistol, J; Gómez, L; Lambruschini, N; Ormazabal, A; Pérez-Dueñas, B; Vilaseca, MA1
Ritchie, JW; Taylor, PM1
Dolan, G; Godin, C1
Araki, M1
Belzecka, K; Jakubiec, A; Puzyńska, L1
Kerr, GR; Waisman, HA1
Tamimie, HS1
Anderson, JA; Bruhl, H; Doeden, D; Michaels, AJ1
Fernstrom, JD1
Benevenga, NJ; Steele, RD1
Aragon, MC; Gimenez, C; Herrero, E; Valdivieso, F1
Lipovac, K; Zanić-Grubisić, T1
Ambrus, CM; Barren, E; Horvath, C; Kalghatgi, K; Karakousis, CP; Sharma, SD1
Cannon-Spoor, HE; DeLisi, LE; Neckers, LM; Potkin, SG; Wyatt, RJ1
Corbeel, L1
Hüther, G; Lane, JD; Neuhoff, V; Piel, N1
Bührdel, P; Grimm, U; Knapp, A; Wässer, S; Wulff, K1
Byrd, D; Olek, K; Wardenbach, P1
Curtius, HC; Farner, H; Rey, F1
Antonioli, C; Gambaro, G; Moffa, M; Moretto, S; Tagliaro, F; Tatò, L; Valentini, R1
Wapnir, RA1
Milovanović, DD; Milovanović, L; Vranjesević, D1
Bjørgo, E; de Carvalho, RM; Flatmark, T1
Bühlen, E; Bühner, R; Byrd, DJ; Kochen, W1
Brase, DA; Loh, HH1
Brown, WJ; Copenhaver, JH; Gunter, R; Schalock, RL1
Airaksinen, MM; MacDonald, EJ; Marvola, M; Turakka, H1
Luchins, D1
Christensen, HN1
Brandt, IK; Elsas, LJ; Jackson, CE; Nance, CS; Nance, WE; Paul, TD1
Levy, HL1
Lifehitz, F; Moak, SA; Wapnir, RA1
Borkowska, I1
Brandt, IK; Elsas, LJ; Jackson, CE; Mamunes, P; Nance, CS; Nance, WE; Paul, TD1
Beaff, D; Bielinski, S; Kirschenbaum, B; Lasser, AE; Schultz, BC1
Andersen, AE; Avins, L1
Blair, JA; Green, A; Leeming, RJ; Raine, DN1
Cabalska, B; Rowecka-Trzebicka, K; Skiendzielewska, A; Stachów, A1
Bührlen, E; Byrd, DJ; Kochen, W1
Brune, GG; Pflughaupt, KW1
Kochen, W1
Byrd, DJ; Kochen, W; Schürrle, L; Trefz, FK1
Byrd, DJ; Kochen, W; Trefz, FK1
Bock, HG; Crawford, LV; Hill, RH; Philen, RM; Springer, MA1
Kakinuma, H; Sato, H; Tsuchiya, M; Yokoyama, Y1
Huether, G1
Gehrmann, J; Neuhoff, V; Schott, K1
Lou, H1
Dembure, P; Elsas, L; Freides, D; Halminski, M; Krause, W; McDonald, L; Salvo, R1
Jablonska, S; Stachow, A; Suffczynska, M1
Chamove, AS; Harlow, HF; Kerr, GR1
Attal, C; Boisse, J; Charpentier, C; Lévy, H; Mozziconacci, P1
Pare, CM1
Marsh, RW1
Kniazev, IuA; Vakhrusheva, LL1
Carver, MJ; Copenhaver, JH; Vacanti, JP1
Cabalska, B; Duczyńska, N1
Brissaud, HE1
Haworth, C; Walmsley, TA1
Mandell, AJ; Spooner, CE1
Lifshitz, F; Wapnir, RA1
Byrd, DJ; Kochen, W; Schürrle, L1
McKean, CM1
Jablonska, S; Stachow, A1
Bühner, R; Bührlen, E; Byrd, D; Kochen, W1
Lees, GJ; Weiner, N1
Tabolin, VA1
Oldendorf, WH1
Bozkowa, K; Cabalska, B; Duczyńska, N1
Alpers, DH; Thier, SO1
Menne, F1
Hirano, S1
Hsia, DY1
Koe, BK; Weissman, A1
Podhradská, O1
Bühner, R; Kochen, W; Otting, W1
Berman, JL; Hsia, DY; Justice, P1
Bunting, R; Diamond, S; Hansen, S; Perry, TL; Tischler, B1
Kahn, LI1
Clayton, BE; Heeley, AF; Heeley, M1
Losiowski, Z1
Jepson, JB; Smith, HG; Smith, WR1
Jéquier, E1
Colombo, JP; Humbel, R; Rossi, E; Vassella, F1
Gershon, ES; Shader, RI1
Heeley, AF1
Bessman, SP; Wapnir, RA1
Charpentier, C; Leluc, R; Lemonnier, A1
Attal, C; Boisse, J; Leluc, R; Mozziconacci, P1
Drummond, KN; Good, RA; Michael, AF1
Anderson, JA; Yarbro, MT1
Frisone, L1

Reviews

12 review(s) available for tryptophan and BH4 Deficiency

ArticleYear
Possible role of tryptophan metabolism along the microbiota-gut-brain axis on cognitive & behavioral aspects in Phenylketonuria.
    Pharmacological research, 2023, Volume: 197

    Topics: Brain-Gut Axis; Child; Cognition; Humans; Phenylketonurias; Quality of Life; Tryptophan

2023
Tissue uptake of thyroid hormone by amino acid transporters.
    Best practice & research. Clinical endocrinology & metabolism, 2007, Volume: 21, Issue:2

    Topics: Acute Disease; Adipose Tissue; Amino Acid Transport Systems; Animals; Biological Transport; Blood-Brain Barrier; Humans; Models, Biological; Organ Specificity; Phenylketonurias; Placenta; Thyroid Hormones; Tryptophan

2007
Role of precursor availability in control of monoamine biosynthesis in brain.
    Physiological reviews, 1983, Volume: 63, Issue:2

    Topics: Animals; Appetite; Blood Pressure; Brain; Brain Chemistry; Catecholamines; Diabetes Mellitus; Diet; Growth Hormone; Humans; Liver Cirrhosis; Methyldopa; Pain; Phenylketonurias; Protein Binding; Rats; Serotonin; Serum Albumin; Tryptophan; Tryptophan Hydroxylase; Tyrosine; Tyrosine 3-Monooxygenase

1983
Adverse effects of excessive consumption of amino acids.
    Annual review of nutrition, 1984, Volume: 4

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Brain; Cattle; Diet; Disease Models, Animal; Ethionine; Fenclonine; Glycine; Growth Disorders; Histidine; Humans; Methionine; Nerve Tissue Proteins; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Pneumonia, Atypical Interstitial, of Cattle; Serine; Stereoisomerism; Sulfhydryl Compounds; Tissue Distribution; Tryptophan; Tyrosine; Vitamin A

1984
Biogenic amines and affective disorders. A critical analysis.
    International pharmacopsychiatry, 1976, Volume: 11, Issue:3

    Topics: 5-Hydroxytryptophan; Affective Symptoms; Antidepressive Agents, Tricyclic; Biogenic Amines; Bipolar Disorder; Brain Chemistry; Clinical Trials as Topic; Depression; Electroconvulsive Therapy; Fenclonine; Fusaric Acid; Humans; Levodopa; Lithium; Methyldopa; Methyltyrosines; Methysergide; Monoamine Oxidase Inhibitors; Phenylketonurias; Reserpine; Tryptophan

1976
Developments in amino acid transport, illustrated for the blood-brain barrier.
    Biochemical pharmacology, 1979, Jul-01, Volume: 28, Issue:13

    Topics: Amino Acids; Animals; Biological Transport; Blood Proteins; Blood-Brain Barrier; Brain; Humans; Phenylketonurias; Protein Binding; Tryptophan

1979
[Metabolism of tryptophan and its significance in pathology of childhood].
    Pediatriia, 1972, Volume: 51, Issue:2

    Topics: Child, Preschool; Cushing Syndrome; Diabetes Mellitus, Type 1; Down Syndrome; Female; Hartnup Disease; Hodgkin Disease; Humans; Infant; Pediatrics; Phenylketonurias; Tryptophan; Vitamin B 6 Deficiency

1972
Psychochemical research studies in man.
    Science (New York, N.Y.), 1968, Dec-27, Volume: 162, Issue:3861

    Topics: Animals; Behavior; Bipolar Disorder; Brain; Brain Chemistry; Depression; Dihydroxyphenylalanine; Hallucinogens; Hartnup Disease; Humans; Imipramine; Metabolism, Inborn Errors; Models, Neurological; Neurons; Neurotransmitter Agents; Norepinephrine; Phenylketonurias; Psychopharmacology; Sleep, REM; Tryptophan

1968
Disorders of intestinal transport of amino acids.
    American journal of diseases of children (1960), 1969, Volume: 117, Issue:1

    Topics: Amino Acids; Arginine; Carbohydrate Metabolism, Inborn Errors; Cystinuria; Fructose; Galactosemias; Genes, Recessive; Glycine; Hartnup Disease; Humans; Intestinal Absorption; Lysine; Methionine; Phenylketonurias; Proline; Renal Tubular Transport, Inborn Errors; Tryptophan

1969
[Feeble mindedness caused by genetic disorders of amino acid metabolism].
    Hippokrates, 1968, May-31, Volume: 39, Issue:10

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; Hydroxyproline; Intellectual Disability; Lysine; Maple Syrup Urine Disease; Methionine; Phenylketonurias; Proline; Sarcosine; Succinates; Transferases; Tryptophan; Valine

1968
[Experimental hyperphenylalanemia].
    Tanpakushitsu kakusan koso. Protein, nucleic acid, enzyme, 1969, Volume: 14, Issue:4

    Topics: Amino Acids; Animals; Behavior, Animal; Brain; Humans; Liver; Phenylalanine; Phenylketonurias; Serotonin; Tryptophan

1969
Biochemical factors in mental retardation.
    Proceedings of the annual meeting of the American Psychopathological Association, 1967, Volume: 56

    Topics: 5-Hydroxytryptophan; Animals; Brain Chemistry; Diet Therapy; Female; Guinea Pigs; Haplorhini; Humans; Infant; Intellectual Disability; Intelligence Tests; Male; Metabolism, Inborn Errors; Monoamine Oxidase; Phenylalanine; Phenylketonurias; Rats; Serotonin; Tryptophan; Tyrosine

1967

Trials

5 trial(s) available for tryptophan and BH4 Deficiency

ArticleYear
Metabolomic changes demonstrate reduced bioavailability of tyrosine and altered metabolism of tryptophan via the kynurenine pathway with ingestion of medical foods in phenylketonuria.
    Molecular genetics and metabolism, 2017, Volume: 121, Issue:2

    Topics: Adolescent; Adult; Amino Acids; Biological Availability; Caseins; Catecholamines; Cross-Over Studies; Diet; Female; Food, Formulated; Gastrointestinal Microbiome; Humans; Kynurenine; Male; Melatonin; Metabolic Networks and Pathways; Metabolomics; Middle Aged; Peptide Fragments; Phenylketonurias; Prebiotics; Serotonin; Tryptophan; Tyrosine; Young Adult

2017
Melatonin and dopamine as biomarkers to optimize treatment in phenylketonuria: effects of tryptophan and tyrosine supplementation.
    The Journal of pediatrics, 2014, Volume: 165, Issue:1

    Topics: Adult; Amino Acids, Neutral; Biomarkers; Chromatography, High Pressure Liquid; Cross-Over Studies; Dietary Supplements; Dopamine; Double-Blind Method; Female; Humans; Male; Melatonin; Middle Aged; Phenylketonurias; Serotonin; Treatment Outcome; Tryptophan; Tyrosine; Young Adult

2014
Large neutral amino acid therapy and phenylketonuria: a promising approach to treatment.
    Molecular genetics and metabolism, 2003, Volume: 79, Issue:2

    Topics: Adult; Amino Acids; Brain; Diet; Dose-Response Relationship, Drug; Female; Humans; Magnetic Resonance Spectroscopy; Male; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

2003
Biogenic amines and affective disorders. A critical analysis.
    International pharmacopsychiatry, 1976, Volume: 11, Issue:3

    Topics: 5-Hydroxytryptophan; Affective Symptoms; Antidepressive Agents, Tricyclic; Biogenic Amines; Bipolar Disorder; Brain Chemistry; Clinical Trials as Topic; Depression; Electroconvulsive Therapy; Fenclonine; Fusaric Acid; Humans; Levodopa; Lithium; Methyldopa; Methyltyrosines; Methysergide; Monoamine Oxidase Inhibitors; Phenylketonurias; Reserpine; Tryptophan

1976
Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man.
    The Journal of clinical investigation, 1985, Volume: 75, Issue:1

    Topics: Adolescent; Adult; Biological Transport; Brain; Child; Clinical Trials as Topic; Dopamine; Female; Humans; Kidney Tubules; Male; Neuropsychological Tests; Phenylalanine; Phenylketonurias; Serotonin; Tryptophan; Tyrosine

1985

Other Studies

101 other study(ies) available for tryptophan and BH4 Deficiency

ArticleYear
Tryptophan metabolism in phenylketonuria: A French adult cohort study.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:5

    Topics: Adult; Blood Chemical Analysis; Chromatography, Liquid; Female; France; Humans; Male; Phenylketonurias; Prospective Studies; Tandem Mass Spectrometry; Tryptophan

2020
[Tryptophan metabolism: utility of plasmatic assay in phenylketonuria, a study in 6 adult patients].
    Annales de biologie clinique, 2018, 04-01, Volume: 76, Issue:2

    Topics: Adult; Blood Chemical Analysis; Case-Control Studies; Chromatography, Liquid; Female; Humans; Male; Metabolome; Middle Aged; Phenylketonurias; Predictive Value of Tests; Reference Values; Tandem Mass Spectrometry; Tryptophan; Young Adult

2018
Biallelic Mutations in DNAJC12 Cause Hyperphenylalaninemia, Dystonia, and Intellectual Disability.
    American journal of human genetics, 2017, 02-02, Volume: 100, Issue:2

    Topics: Alleles; Amino Acid Sequence; Biopterins; Case-Control Studies; Dopamine; Dystonia; Exons; Female; Fibroblasts; Gene Deletion; Genome-Wide Association Study; HSP70 Heat-Shock Proteins; Humans; Intellectual Disability; Male; Pedigree; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Repressor Proteins; Serotonin; Tryptophan; Tryptophan Hydroxylase; Tyrosine; Tyrosine 3-Monooxygenase

2017
Genetic diversity within the R408W phenylketonuria mutation lineages in Europe.
    Human mutation, 2003, Volume: 21, Issue:4

    Topics: Amino Acid Substitution; Arginine; Europe; Founder Effect; Genetic Testing; Genetic Variation; Humans; Microsatellite Repeats; Minisatellite Repeats; Mutation; Phenylketonurias; Tryptophan

2003
Relationship of serum phenylalanine levels and ability of phenylketonurics to hydroxylate tryptophan.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1959, Volume: 100, Issue:3

    Topics: Biochemical Phenomena; Humans; Hydroxylation; Phenylalanine; Phenylketonurias; Tryptophan

1959
Indicanuria in phenylketonuria.
    Metabolism: clinical and experimental, 1960, Volume: 9

    Topics: Humans; Indican; Phenylketonurias; Tryptophan

1960
[Hereditary enzyme defects of amino acid metabolism].
    Ergebnisse der inneren Medizin und Kinderheilkunde, 1962, Volume: 18

    Topics: Albinism; Alkaptonuria; Amino Acids; Humans; Maple Syrup Urine Disease; Metabolic Diseases; Phenylketonurias; Proteins; Tryptophan; Tyrosine

1962
[Changes in tryptophan metabolism in phenylketonuria, hypsarrhythmia, mongolism and various infantile brain diseases].
    Minerva pediatrica, 1962, Dec-15, Volume: 14

    Topics: Brain Diseases; Down Syndrome; Humans; Phenylketonurias; Spasms, Infantile; Tryptophan

1962
[PHENYLKETONURIA. APROPOS OF A FAMILIAL CASE].
    Pediatrie, 1963, Volume: 18

    Topics: Blood Chemical Analysis; Body Fluids; Diet; Diet Therapy; Electroencephalography; Genetics, Medical; Humans; Infant; Iproniazid; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Pyridoxine; Tryptophan; Urine

1963
EXCRETION OF INDOLYL ACIDS IN PHENYLKETONURIC MONKEYS.
    Nature, 1963, Oct-05, Volume: 200

    Topics: Animals; Body Fluids; Haplorhini; Humans; Indoleacetic Acids; Indoles; Lactates; Metabolism; Phenylalanine; Phenylketonurias; Pyruvates; Research; Tryptophan; Urine

1963
DEFECTIVE 5-HYDROXYLATION OF TRYPTOPHAN IN PHENYLKETONURIA.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1964, Volume: 115

    Topics: Blood Chemical Analysis; Body Fluids; Humans; Hydroxyindoleacetic Acid; Hydroxylation; Indoleacetic Acids; Indoles; Metabolism; Phenylalanine; Phenylketonurias; Serotonin; Tryptamines; Tryptophan; Urine

1964
[ENZYMOPATHIES AND NUTRITION].
    Zeitschrift fur arztliche Fortbildung, 1964, Feb-15, Volume: 58

    Topics: Agammaglobulinemia; Celiac Disease; Diet; Diet Therapy; Enzymes; Genetics, Medical; Hepatolenticular Degeneration; Humans; Metabolic Diseases; Phenylketonurias; Sprue, Tropical; Tryptophan; Vitamin B 6 Deficiency

1964
DETERMINATION OF FREE AND TOTAL INDOLE-3-ACETIC ACID AND OF THE INDOLE INDEX.
    Clinical chemistry, 1964, Volume: 10

    Topics: Friedreich Ataxia; Humans; Indoleacetic Acids; Indoles; Intestines; Kynurenine; Metabolism; Myotonia; Phenylketonurias; Poisoning; Research; Schizophrenia; Tryptophan; Urine

1964
[SEROTONIN AND 5-HYDROXYINDOLACETIC ACID IN THE NEWBORN INFANT].
    Minerva pediatrica, 1964, Apr-28, Volume: 16

    Topics: Anemia; Anemia, Aplastic; Cardiovascular System; Gastrointestinal Tract; Humans; Hydroxyindoleacetic Acid; Indoleacetic Acids; Infant; Infant, Newborn; Infant, Newborn, Diseases; Metabolic Diseases; Metabolism; Nervous System; Pellagra; Phenylketonurias; Physiology; Renal Aminoacidurias; Respiratory System; Serotonin; Tryptophan

1964
SOME BIOCHEMICAL LESSONS TO BE LEARNED FROM PHENYLKETONURIA.
    The Journal of pediatrics, 1964, Volume: 64

    Topics: Glutamates; Humans; Indican; Indoleacetic Acids; Indoles; Learning; Metabolism; Phenylacetates; Phenylalanine; Phenylketonurias; Pterins; Serotonin; Tryptophan; Tyrosine

1964
[CHANGES IN THE TRYPTOPHAN METABOLISM IN PHENYLKETONURIA].
    Helvetica paediatrica acta, 1964, Volume: 19

    Topics: Amino Acids; Blood; Body Fluids; Child; Humans; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine; Urine

1964
ON THE MECHANISM OF THE BRAIN SEROTONIN DEPLETION IN EXPERIMENTAL PHENYLKETONURIA.
    The Journal of biological chemistry, 1965, Volume: 240

    Topics: 5-Hydroxytryptophan; Adrenal Glands; Ascorbic Acid; Blood; Brain; Brain Chemistry; Carboxy-Lyases; Cholinesterases; Corticosterone; Dopamine; Glutamates; Liver; Metabolism; Mixed Function Oxygenases; Norepinephrine; Phenylalanine; Phenylketonurias; Rats; Research; Serotonin; Transaminases; Tryptophan; Tyrosine

1965
[RELATIONSHIP BETWEEN METABOLISM OF TRYPTOPHAN AND PYRIDOXIN IN PHENYLKETONURIA].
    Minerva pediatrica, 1965, Feb-11, Volume: 17

    Topics: Humans; Metabolism; Phenylketonurias; Pyridoxine; Tryptophan

1965
Tryptophan metabolism in phenylketonurics.
    JAMA, 1961, Dec-02, Volume: 178

    Topics: Phenylketonurias; Tryptophan

1961
Pathologic and immunohistochemical findings in hypothalamic and mesencephalic regions in the pah(enu2) mouse model for phenylketonuria.
    Pediatric research, 2005, Volume: 58, Issue:2

    Topics: Animals; Catecholamines; CD11b Antigen; Disease Models, Animal; Dopamine; Female; Gene Transfer Techniques; Genotype; Heterozygote; Homozygote; Hypothalamus; Immunohistochemistry; Macrophages; Male; Mesencephalon; Mice; Mice, Mutant Strains; Neurons; Nitric Oxide Synthase; Nitric Oxide Synthase Type II; Phenylketonurias; Substantia Nigra; Time Factors; Tryptophan; Tyrosine

2005
Platelet serotonin concentrations in PKU patients under dietary control and tetrahydrobiopterin treatment.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:6

    Topics: Adolescent; Adult; Biopterins; Blood Platelets; Child; Child, Preschool; Chromatography, High Pressure Liquid; Diet; Female; Humans; Infant; Male; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Serotonin; Tryptophan; Tryptophan Hydroxylase

2005
Tryptophan metabolism in normal and phenylketonuric rats.
    Biochimica et biophysica acta, 1966, Dec-28, Volume: 130, Issue:2

    Topics: Animals; Carbon Dioxide; Carbon Isotopes; Humans; Indoleacetic Acids; Injections, Intravenous; Kynurenine; Male; Phenylalanine; Phenylketonurias; Rats; Tryptophan; Urea

1966
[Neuropsychiatric study on the effects of low-phenylalanine diet in phenylketonuria. 2. Experiments on the metabolism associated with the central nervous system disorder of phenylketonuria].
    Nihon Shonika Gakkai zasshi. Acta paediatrica Japonica, 1966, Volume: 70, Issue:11

    Topics: Child; Child, Preschool; Cyproheptadine; Diet Therapy; Female; Glutamine; Humans; Hydroxyindoleacetic Acid; Infant; Male; Phenylalanine; Phenylketonurias; Tryptophan

1966
The effect of phenylalanine administration on the activities of phenylalanine hydroxylase, some aminotransferases and decarboxylases in adult rats.
    Acta biochimica Polonica, 1967, Volume: 14, Issue:2

    Topics: Animals; Brain; Carboxy-Lyases; Chromatography, Paper; Dopa Decarboxylase; Female; Humans; Injections, Intraperitoneal; Kidney; Liver; Male; Mixed Function Oxygenases; Phenylalanine; Phenylketonurias; Rats; Transaminases; Tryptophan; Tyrosine

1967
Dietary induction of hyperphenylalaninemia in the rat.
    The Journal of nutrition, 1967, Volume: 92, Issue:1

    Topics: Animals; Body Weight; Diet; Female; Growth; Humans; Liver; Mortality; Oxidoreductases; Phenylalanine; Phenylketonurias; Pregnancy; Pregnancy, Animal; Rats; Tryptophan; Tyrosine

1967
Influence of niacin and L-tryptophan on the growth depressive performance of chicks fed high levels of L-phenylalanine and L-methionine.
    Life sciences, 1967, Mar-15, Volume: 6, Issue:6

    Topics: Animals; Body Weight; Diet; Growth; Homocystinuria; Humans; Methionine; Nicotinic Acids; Phenylalanine; Phenylketonurias; Poultry; Tryptophan

1967
Tryptophan oxidation in phenylketonuria.
    Pediatric research, 1967, Volume: 1, Issue:5

    Topics: Adolescent; Child; Child, Preschool; Female; Humans; Indoleacetic Acids; Ketones; Male; Oxidation-Reduction; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Tryptophan

1967
Inhibition by L-phenylalanine of tryptophan transport by synaptosomal plasma membrane vesicles: implications in the pathogenesis of phenylketonuria.
    Journal of inherited metabolic disease, 1983, Volume: 6, Issue:1

    Topics: Animals; Biological Transport; Biological Transport, Active; Brain; Humans; Kinetics; Male; Phenylalanine; Phenylketonurias; Rats; Rats, Inbred Strains; Synaptic Membranes; Synaptosomes; Tryptophan

1983
Disturbances of amino acid transport in rats with experimental hyperphenylalaninaemia.
    Journal of inherited metabolic disease, 1981, Volume: 4, Issue:2

    Topics: Amino Acids; Animals; Cerebral Cortex; Disease Models, Animal; Fenclonine; Glycine; Humans; Leucine; Liver; Phenylalanine; Phenylketonurias; Rats; Tryptophan

1981
Determination of aromatic amino acids by ion-pair reversed-phase liquid chromatography in human sera from healthy and phenylketonuric individuals.
    Research communications in chemical pathology and pharmacology, 1984, Volume: 45, Issue:2

    Topics: Amino Acids; Chromatography, Liquid; Histidine; Humans; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

1984
Plasma phenylalanine, tyrosine, and tryptophan in schizophrenia.
    Archives of general psychiatry, 1983, Volume: 40, Issue:7

    Topics: Administration, Oral; Adult; Heterozygote; Humans; Infusions, Parenteral; Phenylalanine; Phenylketonurias; Schizophrenia; Tryptophan; Tyrosine

1983
[Problems posed by maternal phenylketonuria].
    Archives francaises de pediatrie, 1983, Volume: 40 Suppl 1

    Topics: Brain; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Phenylalanine; Phenylketonurias; Pregnancy; Pregnancy Complications; Tryptophan; Tyrosine

1983
Impaired permeability of the blood-cerebrospinal fluid barrier in hyperphenylalaninaemia.
    Neuropediatrics, 1982, Volume: 13, Issue:2

    Topics: 5-Hydroxytryptophan; Animals; Biological Transport, Active; Blood-Brain Barrier; Brain Chemistry; Cattle; Choroid Plexus; Humans; In Vitro Techniques; Permeability; Phenylalanine; Phenylketonurias; Rats; Serotonin; Tryptophan

1982
[Tryptophan metabolism in infants with phenylketonuria].
    Padiatrie und Grenzgebiete, 1982, Volume: 21, Issue:4

    Topics: Humans; Infant; Phenylketonurias; Tryptophan

1982
[Excretion of transamination products in hyperphenylalaninemia (author's transl)].
    Klinische Wochenschrift, 1980, Feb-01, Volume: 58, Issue:3

    Topics: Adult; Biogenic Amines; Female; Histidine; Humans; Intellectual Disability; Middle Aged; Phenylalanine; Phenylketonurias; Transaminases; Tryptophan

1980
In vivo studies of the tryptophan-5-hydroxylase system. Quantitation of serotonin and tryptamine using gas chromatography-mass fragmentography.
    Journal of chromatography, 1980, Oct-31, Volume: 199

    Topics: Biopterins; Gas Chromatography-Mass Spectrometry; Humans; Phenylketonurias; Serotonin; Tryptophan; Tryptophan Hydroxylase

1980
Capillary zone electrophoresis determination of phenylalanine in serum: a rapid, inexpensive and simple method for the diagnosis of phenylketonuria.
    Electrophoresis, 1994, Volume: 15, Issue:1

    Topics: Capillary Action; Electrophoresis; Humans; Phenylalanine; Phenylketonurias; Quality Control; Tryptophan; Tyrosine

1994
L-tryptophan in maternal phenylketonuria.
    The American journal of clinical nutrition, 1998, Volume: 68, Issue:6

    Topics: Female; Humans; Phenylalanine; Phenylketonurias; Pregnancy; Pregnancy Complications; Tryptophan; Tyrosine

1998
Serum tryptophan to large neutral amino acid ratio and urinary tryptophan in three patients with phenylketonuria in a family. A clinical and biochemical study.
    Advances in experimental medicine and biology, 1999, Volume: 467

    Topics: Adult; Amino Acids; Child; Child, Preschool; Female; Humans; Male; Middle Aged; Nuclear Family; Pedigree; Phenylketonurias; Reference Values; Tryptophan

1999
A comparison of kinetic and regulatory properties of the tetrameric and dimeric forms of wild-type and Thr427-->Pro mutant human phenylalanine hydroxylase: contribution of the flexible hinge region Asp425-Gln429 to the tetramerization and cooperative subs
    European journal of biochemistry, 2001, Volume: 268, Issue:4

    Topics: Enzyme Stability; Escherichia coli; Humans; Kinetics; Mutagenesis, Site-Directed; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Protein Structure, Quaternary; Recombinant Proteins; Spectrometry, Fluorescence; Transfection; Trypsin; Tryptophan

2001
[Studies on tryptophan metabolism in untreated phenylketonuric patients].
    Zeitschrift fur klinische Chemie und klinische Biochemie, 1975, Volume: 13, Issue:1

    Topics: Acetophenones; Adolescent; Adult; Biotransformation; Child; Child, Preschool; Chromatography, Ion Exchange; Chromatography, Thin Layer; Female; Humans; Kynurenic Acid; Kynurenine; Male; Mass Spectrometry; Niacinamide; Nicotinic Acids; ortho-Aminobenzoates; Phenylalanine; Phenylketonurias; Quinolines; Tryptophan; Xanthurenates

1975
Possible role of 5-hydroxyptamine in minimal brain dysfunction.
    Life sciences, 1975, Apr-01, Volume: 16, Issue:7

    Topics: Amphetamine; Animal Nutritional Physiological Phenomena; Animals; Attention Deficit Disorder with Hyperactivity; Behavior, Animal; Child; Drug Interactions; Fenclonine; Humans; Hyperkinesis; Norepinephrine; Phenylketonurias; Serotonin; Tryptophan

1975
Model phenylketonuria (PKU) in the albino rat: behaviroal, biochemical, and neuroanatomical effects.
    Journal of comparative and physiological psychology, 1975, Volume: 89, Issue:6

    Topics: Animals; Axons; Body Weight; Disease Models, Animal; Emotions; Fenclonine; Humans; Learning; Liver; Motor Activity; Myelin Sheath; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Rats; Social Behavior; Tryptophan

1975
Protective effect of tryptophan and 5-hydroxytryptophan on experimental phenylketonuria induced with phenylalanine+ p-cholorophenylalanine in rats.
    Medical biology, 1975, Volume: 53, Issue:6

    Topics: 5-Hydroxytryptophan; Animals; Behavior, Animal; Body Weight; Brain; Female; Fenclonine; Humans; Hydroxyindoleacetic Acid; Male; Motor Activity; Norepinephrine; Phenylalanine; Phenylketonurias; Rats; Serotonin; Tryptophan

1975
Linkage analysis using heterozygote detection in phenylketonuria.
    Clinical genetics, 1979, Volume: 16, Issue:4

    Topics: Chromosome Mapping; Ethnicity; Female; Genetic Carrier Screening; Genetic Linkage; Heterozygote; Humans; Male; Pedigree; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

1979
Treatment of phenylketonuria.
    Progress in clinical and biological research, 1979, Volume: 34

    Topics: Adult; Child; Child Behavior Disorders; Dihydroxyphenylalanine; Female; Humans; Infant, Newborn; Male; Phenylalanine; Phenylketonurias; Pregnancy; Pregnancy Complications; Time Factors; Tryptophan

1979
Decreased foetal amino acid uptake, brain pyruvate kinase and intrauterine damage in maternal PKU.
    Nature, 1977, Feb-17, Volume: 265, Issue:5595

    Topics: Amino Acids; Animals; Brain; Female; Fetus; Hexokinase; Humans; Maternal-Fetal Exchange; Phenylalanine; Phenylketonurias; Pregnancy; Pregnancy Complications; Pyruvate Kinase; Rats; Succinate Dehydrogenase; Tryptophan; Tyrosine

1977
[Metabolism of amino acids and brain proteins in phenylketonuria].
    Postepy biochemii, 1979, Volume: 25, Issue:2

    Topics: Animals; Brain; Catecholamines; Chemical Phenomena; Chemistry; Glycolysis; Humans; Nerve Tissue Proteins; Phenylalanine; Phenylketonurias; Pyridoxine; Serotonin; Tryptophan; Tyrosine

1979
Phenylketonuria heterozygote detection in families with affected children.
    American journal of human genetics, 1978, Volume: 30, Issue:3

    Topics: Ethnicity; Female; Genetic Counseling; Heterozygote; Humans; Indiana; Male; Michigan; Pedigree; Phenylalanine; Phenylketonurias; Pregnancy; Selection, Genetic; Sex Factors; Tryptophan; Tyrosine

1978
Phenylketonuria and scleroderma.
    Archives of dermatology, 1978, Volume: 114, Issue:8

    Topics: Child; Humans; Intellectual Disability; Male; Phenylalanine; Phenylketonurias; Scleroderma, Localized; Tryptophan

1978
Lowering brain phenylalanine levels by giving other large neutral amino acids. A new experimental therapeutic approach to phenylketonuria.
    Archives of neurology, 1976, Volume: 33, Issue:10

    Topics: Amino Acids; Animals; Brain Chemistry; Dose-Response Relationship, Drug; Drug Combinations; Humans; Isoleucine; Leucine; Phenylalanine; Phenylketonurias; Rats; Threonine; Tryptophan; Tyrosine; Valine

1976
Biopterin derivatives in normal and phenylketonuric patients after oral loads of L-phenylalanine, L-tyrosine, and L-tryptophan.
    Archives of disease in childhood, 1976, Volume: 51, Issue:10

    Topics: Administration, Oral; Adolescent; Adult; Biopterins; Child, Preschool; Chromatography, Thin Layer; Humans; Infant; Infant, Newborn; Phenylalanine; Phenylketonurias; Pteridines; Tryptophan; Tyrosine

1976
[Scleroderma-like skin and muscle changes in children with phenylketonuria].
    Pediatria polska, 1975, Volume: 50, Issue:12

    Topics: Age Factors; Child; Child, Preschool; Diagnosis, Differential; Female; Humans; Infant; Male; Muscular Diseases; Phenylketonurias; Scleroderma, Localized; Skin Diseases; Tryptophan

1975
Tryptophan metabolism in untreated phenylketonuria.
    Acta vitaminologica et enzymologica, 1975, Volume: 29, Issue:1-6

    Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Humans; Indoles; Kynurenine; Phenylketonurias; Tryptophan

1975
Tryptophan metabolism in nervous disease.
    Acta vitaminologica et enzymologica, 1975, Volume: 29, Issue:1-6

    Topics: Acute Disease; Child; Hartnup Disease; Hepatolenticular Degeneration; Humans; Infant; Phenylketonurias; Porphyrias; Tryptophan

1975
Perspectives for ISTRY: aims and alternatives.
    Acta vitaminologica et enzymologica, 1975, Volume: 29, Issue:1-6

    Topics: Humans; Indoles; Methods; Phenylketonurias; Tryptophan

1975
A study of urinary tryptophan metabolites in relation to the phenylalanine content of semi-synthetic diets in a patient with phenylketonuria.
    Acta vitaminologica et enzymologica, 1975, Volume: 29, Issue:1-6

    Topics: Adult; Diet; Humans; Male; Phenylalanine; Phenylketonurias; Tryptophan

1975
[Point of micropreparative high pressure liquid chromatography and thin-layer chromatography for the identification of indole compounds in human plasma (author's transl)].
    Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie, 1976, Volume: 14, Issue:2

    Topics: Adolescent; Adult; Benzaldehydes; Child; Chromatography, High Pressure Liquid; Chromatography, Thin Layer; Female; Humans; Indoleacetic Acids; Indoles; Lactates; Male; Mass Spectrometry; Phenylketonurias; Tryptophan; Uremia

1976
Eosinophilia-myalgia syndrome in a child with phenylketonuria.
    Pediatrics, 1992, Volume: 90, Issue:4

    Topics: Child, Preschool; Eosinophilia-Myalgia Syndrome; Food Contamination; Food, Formulated; Humans; Male; Phenylketonurias; Tryptophan

1992
Simultaneous determination of urinary creatinine and aromatic amino acids by cation-exchange chromatography with ultraviolet detection.
    Journal of chromatography, 1991, May-03, Volume: 566, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Cations; Chromatography, High Pressure Liquid; Chromatography, Ion Exchange; Creatinine; Humans; Infant, Newborn; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

1991
The depletion of tryptophan and serotonin in the brain of developing hyperphenylalaninemic rats is abolished by the additional administration of lysine.
    Neurochemical research, 1986, Volume: 11, Issue:12

    Topics: Animals; Brain Chemistry; Hydroxyindoleacetic Acid; Lysine; Male; Phenylalanine; Phenylketonurias; Rats; Rats, Inbred Strains; Serotonin; Tryptophan

1986
Biochemical and developmental features of experimental phenylketonuria induced by L-ethionine in suckling rats.
    Biochemical medicine and metabolic biology, 1989, Volume: 41, Issue:3

    Topics: Adenosine Triphosphate; Animals; Animals, Suckling; Body Weight; Brain Chemistry; Electrophoresis, Gel, Two-Dimensional; Ethionine; Liver; Male; Methionine; Organ Size; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Rats; Rats, Inbred Strains; Tryptophan

1989
Large doses of tryptophan and tyrosine as potential therapeutic alternative to dietary phenylalanine restriction in phenylketonuria.
    Lancet (London, England), 1985, Jul-20, Volume: 2, Issue:8447

    Topics: Drug Therapy, Combination; Humans; Phenylketonurias; Tryptophan; Tyrosine

1985
Skin and muscle indurations in phenylketonuria.
    Archives of dermatology, 1967, Volume: 95, Issue:5

    Topics: Biopsy; Child, Preschool; Chromatography, Paper; Electromyography; Extremities; Humans; Male; Muscles; Muscular Atrophy; Phenylketonurias; Skin; Staining and Labeling; Tryptophan; Urine

1967
Learning in monkeys fed elevated amino acid diets.
    Journal of medical primatology, 1973, Volume: 2, Issue:3

    Topics: Alanine; Amino Acids; Animals; Diet; Discrimination Learning; Female; Food; Glycine; Haplorhini; Histidine; Humans; Learning; Macaca; Monkey Diseases; Phenylalanine; Phenylketonurias; Pregnancy; Problem Solving; Reward; Tryptophan; Tyrosine

1973
[Tryptophan tolerance test in children].
    Annales de pediatrie, 1964, Mar-02, Volume: 11, Issue:3

    Topics: Adolescent; Child; Child, Preschool; Down Syndrome; Hartnup Disease; Humans; Infant; Metabolism, Inborn Errors; Phenylketonurias; Seizures; Tryptophan; Vitamin B Deficiency

1964
5-hydroxyindoles in phenylketonuric and nonphenylketonuric mental defectives.
    Advances in pharmacology, 1968, Volume: 6, Issue:Pt B

    Topics: 5-Hydroxytryptophan; Animals; Biological Transport; Carboxy-Lyases; Child; Down Syndrome; Humans; Hydroxyindoleacetic Acid; Indoles; Intellectual Disability; Phenylketonurias; Rats; Serotonin; Tryptophan

1968
The feasibility of treating Down's disease with a reduced phenylalanine diet.
    The New Zealand medical journal, 1971, Volume: 74, Issue:471

    Topics: Diet Therapy; Down Syndrome; Humans; Infant, Newborn; Phenylalanine; Phenylketonurias; Tryptophan

1971
Experimental maternal hyperphenylalaninemia: disaggregation of fetal brain ribosomes.
    Journal of neurochemistry, 1973, Volume: 21, Issue:2

    Topics: Animals; Brain; Brain Chemistry; Centrifugation, Density Gradient; Female; Fenclonine; Fetus; Humans; Maternal-Fetal Exchange; Phenylalanine; Phenylketonurias; Polyribosomes; Pregnancy; Rats; Tryptophan; Tyrosine

1973
[Phenylketonuria. 3. Evaluation of early treatment].
    Pediatria polska, 1969, Volume: 44, Issue:8

    Topics: Blood Proteins; Child, Preschool; Diet Therapy; Female; Humans; Infant; Male; Phenylalanine; Phenylketonurias; Phosphoric Monoester Hydrolases; Tryptophan; Tyrosine

1969
[Dietetics of amino acid metabolism disorders].
    La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris, 1971, Feb-08, Volume: 47, Issue:7

    Topics: Amino Acid Metabolism, Inborn Errors; Diet Therapy; Glycine; Histidine; Homocystinuria; Humans; Isoleucine; Leucine; Phenylketonurias; Tryptophan; Tyrosine; Valine

1971
A study of the chromatographic behaviour of tryptophan metabolites and related compounds by chromatography on thin layers of silica gel. I. Qualitative separation.
    Journal of chromatography, 1972, Apr-05, Volume: 66, Issue:2

    Topics: Chromatography, Thin Layer; Gels; Humans; Indicators and Reagents; Methods; Phenylketonurias; Silicon Dioxide; Solvents; Tryptophan

1972
Intestinal transport of aromatic amino acids, glucose and electrolytes in a patient with phenylketonuria.
    Clinica chimica acta; international journal of clinical chemistry, 1974, Aug-20, Volume: 54, Issue:3

    Topics: Adult; Biological Transport; Female; Glucose; Humans; Intestinal Mucosa; Male; Molecular Weight; Phenylalanine; Phenylketonurias; Polyethylene Glycols; Potassium; Sodium; Time Factors; Tryptophan; Tyrosine

1974
Proceedings: New aspects of tryptophan metabolism in untreated phenylketonuria and the urinary indole excretion in relation to phenylalanine content of semi-synthetic diets.
    Hoppe-Seyler's Zeitschrift fur physiologische Chemie, 1974, Volume: 355, Issue:10

    Topics: Diet; Humans; Indoles; Phenylalanine; Phenylketonurias; Tryptophan

1974
The effects of high phenylalanine concentrations on serotonin and catecholamine metabolism in the human brain.
    Brain research, 1972, Dec-12, Volume: 47, Issue:2

    Topics: Adolescent; Adult; Amino Acids; Brain; Catecholamines; Chromatography; Dopamine; Humans; Hydroxyindoleacetic Acid; Norepinephrine; Phenylacetates; Phenylalanine; Phenylketonurias; Probenecid; Serotonin; Tryptophan; Tyrosine

1972
[Pseudoscleroderma or scleroderma coexisting with phenylketonuria].
    Annales de dermatologie et de syphiligraphie, 1972, Volume: 99, Issue:3

    Topics: Child; Diagnosis, Differential; Female; Humans; Kynurenine; Phenylketonurias; Scleroderma, Localized; Skin; Tryptophan

1972
[Proceedings: Tryptophan metabolism in untreated phenylketonuria and in vitamin B6 dependent cramps].
    Zeitschrift fur klinische Chemie und klinische Biochemie, 1972, Volume: 10, Issue:4

    Topics: 3-Hydroxyanthranilic Acid; Adolescent; Adult; Carboxylic Acids; Child; Child, Preschool; Humans; Hydroxyquinolines; Intellectual Disability; Kynurenic Acid; Kynurenine; Muscle Cramp; Nicotinic Acids; Phenylalanine; Phenylketonurias; Tryptophan; Vitamin B 6 Deficiency; Xanthurenates

1972
Transaminations between amino acids and keto acids elevated in phenylketonuria and maple syrup urine disease.
    Journal of neurochemistry, 1973, Volume: 20, Issue:2

    Topics: Amino Acids; Animals; Brain Stem; Carbon Isotopes; Cerebellum; Dialysis; Dihydroxyphenylalanine; Humans; Hydrogen-Ion Concentration; In Vitro Techniques; Keto Acids; Ketoglutaric Acids; Kinetics; Liver; Maple Syrup Urine Disease; Phenylketonurias; Pyruvates; Rats; Transaminases; Tritium; Tryptophan

1973
[Metabolic disorders, their early diagnosis and therapy in newborn infants].
    Vestnik Akademii meditsinskikh nauk SSSR, 1973, Volume: 28, Issue:6

    Topics: Adrenal Hyperplasia, Congenital; Amino Acid Metabolism, Inborn Errors; Carbohydrate Metabolism, Inborn Errors; Child, Preschool; Cystic Fibrosis; Fanconi Syndrome; Humans; Hyperbilirubinemia, Hereditary; Immunologic Deficiency Syndromes; Infant; Infant, Newborn; Jaundice, Neonatal; Malabsorption Syndromes; Neurologic Manifestations; Phenylketonurias; Tryptophan

1973
Saturation of blood brain barrier transport of amino acids in phenylketonuria.
    Archives of neurology, 1973, Volume: 28, Issue:1

    Topics: Amino Acids; Animals; Arginine; Blood-Brain Barrier; Brain; Carbon Isotopes; Dihydroxyphenylalanine; Female; Histidine; Humans; Intellectual Disability; Isoleucine; Leucine; Lysine; Male; Methionine; Ornithine; Phenylalanine; Phenylketonurias; Rats; Threonine; Tryptophan; Tyrosine; Valine

1973
[Investigations on the behaviour of tryptophan in children with phenylketonuria (author's transl)].
    Problemy medycyny wieku rozwojowego, 1973, Volume: 3

    Topics: Administration, Oral; Adolescent; Age Factors; Child; Child, Preschool; Chromatography, Paper; Diet Therapy; Humans; Infant; Intestinal Absorption; Phenylalanine; Phenylketonurias; Tryptophan

1973
The pharmacology of para-chlorophenylalanine, a selective depletor of serotonin stores.
    Advances in pharmacology, 1968, Volume: 6, Issue:Pt B

    Topics: Animals; Behavior, Animal; Body Temperature; Brain; Dogs; Haplorhini; Humans; Liver; Male; Mixed Function Oxygenases; Norepinephrine; Pargyline; Phenylalanine; Phenylketonurias; Rats; Serotonin; Sleep; Spleen; Tryptophan

1968
[Therapy of phenylketonuria].
    Casopis lekaru ceskych, 1972, Volume: 111, Issue:8

    Topics: Diet Therapy; Humans; Methionine; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

1972
[Identification of oxindole and 3-indolecarbaldehyde in the urine of patients with untreated phenylketonuria].
    Hoppe-Seyler's Zeitschrift fur physiologische Chemie, 1972, Volume: 353, Issue:7

    Topics: Aldehydes; Carbamates; Chromatography, Thin Layer; Humans; Indoles; Kynurenine; Mass Spectrometry; Nicotinic Acids; Phenylketonurias; Spectrophotometry, Infrared; Spectrophotometry, Ultraviolet; Tryptophan

1972
Effect of vitamin B 6 on blood 5-hydroxytryptamine concentration.
    Annals of the New York Academy of Sciences, 1969, Sep-30, Volume: 166, Issue:1

    Topics: Animals; Animals, Newborn; Birth Weight; Body Weight; Brain; Carboxy-Lyases; Desoxycorticosterone; Diet; Female; Fetus; Humans; In Vitro Techniques; Indoleacetic Acids; Infant; Infant, Newborn; Kidney; Liver; Phenylalanine; Phenylketonurias; Postpartum Period; Pregnancy; Pyridoxine; Rats; Serotonin; Tryptophan; Tyrosine; Umbilical Cord; Vitamin B 6 Deficiency

1969
Glutamine depletion in phenylketonuria. A possible cause of the mental defect.
    The New England journal of medicine, 1970, 04-02, Volume: 282, Issue:14

    Topics: Adult; Amino Acids; Diet Therapy; Female; Glutamine; Humans; Intellectual Disability; Intelligence; Intelligence Tests; Male; Phenylalanine; Phenylketonurias; Tryptophan

1970
From the Clinical Conference St. Louis Children's Hospital. Phenylketonuria. Diagnostic and therapeutic dilemma.
    Clinical pediatrics, 1970, Volume: 9, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Diet Therapy; Female; Humans; Infant; Intellectual Disability; Phenylalanine; Phenylketonurias; Transaminases; Tryptophan; Tyrosine

1970
An investigation of the hyperaminoaciduria in phenylketonuria associated with the feeding of certain commercial low-phenylalanine preparations.
    The British journal of nutrition, 1970, Volume: 24, Issue:2

    Topics: Alanine; Amino Acids; Child; Child, Preschool; Diet Therapy; Female; Humans; Hydro-Lyases; Infant; Male; Metabolic Diseases; Phenylalanine; Phenylketonurias; Proteins; Stereoisomerism; Tryptophan

1970
[Urinary excretion of xanthurenic acid in children with untreated phenylketonuria].
    Pediatria polska, 1971, Volume: 46, Issue:6

    Topics: Child; Child, Preschool; Colorimetry; Humans; Infant; Phenylketonurias; Tryptophan; Xanthurenates

1971
Interconversions of indolic acids by bacteria and rat tissue--possible relevance to Hartnup disorder.
    Clinical science, 1968, Volume: 34, Issue:2

    Topics: Animals; Chromatography, Thin Layer; Electrophoresis; Hartnup Disease; Humans; Indoles; Injections, Intraperitoneal; Intestine, Small; Intubation, Gastrointestinal; Kidney; Liver; Male; Phenylketonurias; Rats; Tryptophan

1968
Tryptophan hydroxylation in phenylketonuria.
    Advances in pharmacology, 1968, Volume: 6, Issue:Pt B

    Topics: Adult; Animals; Carbon Isotopes; Chromatography, Paper; Humans; Hydroxyindoleacetic Acid; Mixed Function Oxygenases; Phenylketonurias; Rats; Tryptophan

1968
[L-tryptophan metabolism in phenylketonuria].
    Helvetica paediatrica acta, 1968, Volume: 23, Issue:1

    Topics: Adolescent; Adult; Child; Diet Therapy; Female; Humans; Indoles; Kynurenine; Male; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

1968
Screening for aminoacidurias in psychiatric inpatients.
    Archives of general psychiatry, 1969, Volume: 21, Issue:1

    Topics: Affective Symptoms; Amino Acid Metabolism, Inborn Errors; Brain Damage, Chronic; Depression; Drug Synergism; Hartnup Disease; Homocystinuria; Hospitals, Psychiatric; Humans; Intellectual Disability; Mass Screening; Massachusetts; Mental Disorders; Methionine; Monoamine Oxidase Inhibitors; Neurotic Disorders; Personality Disorders; Phenylketonurias; Schizophrenia; Substance-Related Disorders; Tryptophan

1969
The effect of pyridoxine on tryptophan metabolism in phenylketonuria.
    Clinical science, 1965, Volume: 29, Issue:3

    Topics: Child; Child, Preschool; Chromatography, Ion Exchange; Diet Therapy; Humans; In Vitro Techniques; Infant; Infant, Newborn; Kynurenine; ortho-Aminobenzoates; Phenylketonurias; Pyridoxine; Tryptophan; Urine; Xanthurenates

1965
Assay of free tryptophan in urine by high-voltage electrophoresis.
    Analytical biochemistry, 1965, Volume: 12, Issue:3

    Topics: Electrophoresis; Humans; In Vitro Techniques; Phenylketonurias; Tryptophan; Urine

1965
[Biochemical and analytical bases of the exploration of phenylalanine metabolism].
    Annales de pediatrie, 1964, Mar-02, Volume: 11, Issue:3

    Topics: Humans; Intellectual Disability; Metabolism, Inborn Errors; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine

1964
["Borderline" forms of phenylketonuria].
    Annales de pediatrie, 1964, Mar-02, Volume: 11, Issue:3

    Topics: Adolescent; Child, Preschool; Humans; Infant; Intellectual Disability; Metabolism, Inborn Errors; Phenylalanine; Phenylketonurias; Serotonin; Tryptophan

1964
Tryptophan metabolism in a patient with phenylketonuria and scleroderma: a proposed explanation of the indole defect in phenylketonuria.
    Canadian Medical Association journal, 1966, Apr-16, Volume: 94, Issue:16

    Topics: Chromatography, Paper; Humans; In Vitro Techniques; Indican; Indoles; Infant; Male; Phenylketonurias; Scleroderma, Systemic; Tryptophan; Urine

1966
L-tryptophan metabolism in phenylketonuria.
    The Journal of pediatrics, 1966, Volume: 68, Issue:6

    Topics: Adolescent; Adult; Blood Chemical Analysis; Child; Child, Preschool; Chromatography; Diet; Feces; Female; Humans; Infant; Male; Phenylalanine; Phenylketonurias; Tryptophan; Tyrosine; Urine

1966
Pyridoxine and phenylketonuria.
    JAMA, 1966, Apr-25, Volume: 196, Issue:4

    Topics: Humans; Phenylketonurias; Pyridoxine; Tryptophan

1966
[Contribution to the study of phenylketonuria].
    Giornale di psichiatria e di neuropatologia, 1966, Volume: 94, Issue:3

    Topics: Adult; Diet Therapy; Erythrocytes, Abnormal; Female; Humans; Male; Middle Aged; Phenylalanine; Phenylketonurias; Tryptophan

1966