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hydroxyindoleacetic acid and BH4 Deficiency

hydroxyindoleacetic acid has been researched along with BH4 Deficiency in 36 studies

(5-hydroxyindol-3-yl)acetic acid : A member of the class of indole-3-acetic acids that is indole-3-acetic acid substituted by a hydroxy group at C-5.

Research Excerpts

ExcerptRelevanceReference
"In a group of 9 patients with classical phenylketonuria (PKU) aged 15-24 years we examined the effect of phenylalanine restricted diet on vigilance, as judged by the continuous visual reaction times, and neurotransmitter synthesis, as judged by cerebrospinal fluid (CSF) homovanillic acid (HVA) and 5-hydroxyindole acetic acid (5-HIAA) levels."2.66Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria. ( Bruhn, P; Gerdes, AM; Lou, HC; Lykkelund, C; Udesen, H, 1987)
" Time of diagnosis, dosage of BH4 and neurotransmitter precursors, folinic acid substitution, and levels of 5-hydroxyindoleacetic acid (5HIAA) and homovanillic acid (HVA) in cerebrospinal fluid (CSF) are essential parameters in the follow-up of patients."1.35Outcome and long-term follow-up of 36 patients with tetrahydrobiopterin deficiency. ( Ballhausen, D; Baumgartner, MR; Blau, N; Fiori, L; Giovannini, M; Hoffmann, GF; Ibel, H; Jäggi, L; Ponzone, A; Porta, F; Santer, R; Schuler, A; Wendel, U; Zurflüh, MR, 2008)
"In a child presenting with malignant phenylketonuria due to dihydrobiopterin synthetase deficiency, the authors studied the cerebrospinal fluid (CSF) homovanillic acid and 5 hydroxyindole acetic acid levels under different circumstances: without treatment; under a treatment with tetrahydrobiopterin used alone at various doses; under a treatment associating BH4, L-dopa, 5 hydroxytryptophan and carbidopa, with increasing doses and varying administration schedules."1.27[Malignant phenylketonuria caused by biopterin synthetase deficiency. Study of neuromediator catabolites in the cerebrospinal fluid during treatment]. ( Boespflug, O; Demeocq, F; Guyon, A; Malpuech, G; Piton, A; Vanlieferinghen, P, 1984)
"Seven phenylketonuria (PKU) patients aged 15-24 years were allowed unrestricted diet for 3 weeks."1.27Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine. ( Christensen, E; Gerdes, AM; Güttler, F; Lou, HC; Lykkelund, C; Nielsen, JB; Rasmussen, V, 1988)
"It is postulated that the seizures and neurological deterioration of the patient were related to a deficiency in the synthesis of biogenic amine neurotransmitters."1.26Biogenic amine synthesis defect in dihydropteridine reductase deficiency. ( Butler, IJ; Koslow, SH, 1977)
"An experimental phenylketonuria-like syndrome was produced in rats by oral administration of 1-phenylalanine (Phe, 500 mg/kg) and dl-p-chloro-phenylalanine (pCPA, 100-125 mg/kg) daily from the 2nd-3rd day of life to the age of 42 days."1.25Protective effect of tryptophan and 5-hydroxytryptophan on experimental phenylketonuria induced with phenylalanine+ p-cholorophenylalanine in rats. ( Airaksinen, MM; MacDonald, EJ; Marvola, M; Turakka, H, 1975)

Research

Studies (36)

TimeframeStudies, this research(%)All Research%
pre-199028 (77.78)18.7374
1990's4 (11.11)18.2507
2000's4 (11.11)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Pascucci, T2
Ventura, R2
Puglisi-Allegra, S2
Cabib, S2
HSIA, DY1
ROWLEY, WA1
HUANG, I1
PERRY, TL1
HANSEN, S1
TISCHLER, B1
HESTRIN, M1
MURTAGH, JJ1
RAPPALLINI, C1
Jäggi, L1
Zurflüh, MR1
Schuler, A1
Ponzone, A2
Porta, F1
Fiori, L1
Giovannini, M1
Santer, R1
Hoffmann, GF2
Ibel, H1
Wendel, U1
Ballhausen, D1
Baumgartner, MR1
Blau, N5
Araki, M1
Malpuech, G1
Guyon, A1
Demeocq, F1
Piton, A1
Boespflug, O1
Vanlieferinghen, P1
de Almeida, IT1
Leandro, PP1
Portela, R1
Cabral, A1
Eusébio, F1
Tasso, T1
Matasovic, A2
Diamond, A1
Ciaramitaro, V1
Donner, E1
Djali, S1
Robinson, MB1
Thöny, B1
Renneberg, A1
Arnold, LA1
Hyland, K1
Cali, F1
Romano, V1
Burlina, AB1
Bonafé, L1
Ferrari, V1
Suppiej, A1
Zacchello, F1
Burlina, AP1
Koslow, SH2
Butler, IJ2
Krumholz, A1
Holtzman, NA1
Kaufman, S2
Gröbe, H1
Bartholome, K1
Milstien, S1
Danks, DM1
Cotton, RG2
Schlesinger, P1
Airaksinen, MM1
Turakka, H1
Marvola, M1
MacDonald, EJ1
Kanabus, P1
Kohsaka, S1
Tsukada, Y1
Heizmann, CW1
Sperl, W1
Korenke, GC1
Smooker, PM1
Lou, HC3
Güttler, F2
Lykkelund, C3
Bruhn, P2
Niederwieser, A3
Huether, G1
Guardamagna, O2
Ferraris, S1
Biasetti, S1
Bracco, G1
Gerdes, AM2
Udesen, H1
Nielsen, JB1
Rasmussen, V1
Christensen, E1
Curtius, HC1
Kierat, L1
Leimbacher, W1
Binkert, F1
Lehmann, H1
Leupold, D1
Pare, CM1
Tu, J1
Partington, MW1
Blatteis, CM1
Billmeier, GJ1
Gilbert, TM1
Costil, J1
Galli, J1
Richardet, JM1
Brissaud, HE1
Loo, YH1
Behbehani, AW1
Quentin, CD1
Schulte, FJ1
Neuhoff, V1
McKean, CM1
Wysokowski, J1
Szonert, M1
Matsuda, I1
Sugai, M1
Arashima, S1
Anakura, M1
Jéquier, E1

Reviews

1 review available for hydroxyindoleacetic acid and BH4 Deficiency

ArticleYear
[5-hydroxyindoleacetic acid and its diagnostic evaluation].
    Polskie Archiwum Medycyny Wewnetrznej, 1967, Volume: 39, Issue:4

    Topics: Brain Neoplasms; Humans; Hydroxyindoleacetic Acid; Phenylketonurias; Serotonin

1967

Trials

1 trial available for hydroxyindoleacetic acid and BH4 Deficiency

ArticleYear
Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria.
    Acta paediatrica Scandinavica, 1987, Volume: 76, Issue:4

    Topics: Adolescent; Adult; Arousal; Clinical Trials as Topic; Dopamine; Double-Blind Method; Female; Homovan

1987

Other Studies

34 other studies available for hydroxyindoleacetic acid and BH4 Deficiency

ArticleYear
Deficits in brain serotonin synthesis in a genetic mouse model of phenylketonuria.
    Neuroreport, 2002, Dec-20, Volume: 13, Issue:18

    Topics: 5-Hydroxytryptophan; Animals; Blood-Brain Barrier; Brain; Disease Models, Animal; Female; Genotype;

2002
The excretion of 5-hydroxyindoleacetic acid in the heterozygous carrier for phenylketonuria.
    Journal of mental deficiency research, 1961, Volume: 5

    Topics: Biological Transport; Humans; Hydroxyindoleacetic Acid; Indoleacetic Acids; Intellectual Disability;

1961
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

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

1964
Outcome and long-term follow-up of 36 patients with tetrahydrobiopterin deficiency.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:3

    Topics: Adolescent; Adult; Biopterins; Child; Dihydropteridine Reductase; Female; Follow-Up Studies; Homovan

2008
[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; Hydroxyindoleaceti

1966
[Malignant phenylketonuria caused by biopterin synthetase deficiency. Study of neuromediator catabolites in the cerebrospinal fluid during treatment].
    Archives francaises de pediatrie, 1984, Volume: 41, Issue:1

    Topics: Alcohol Oxidoreductases; Biopterins; Carbidopa; Follow-Up Studies; Homovanillic Acid; Humans; Hydrox

1984
Tetrahydrobiopterin deficiency in Portugal: results of the screening for hyperphenylalaninemia.
    Advances in experimental medicine and biology, 1993, Volume: 338

    Topics: Adolescent; Biopterins; Child; Child, Preschool; Consanguinity; Female; Homovanillic Acid; Humans; H

1993
An animal model of early-treated PKU.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 1994, Volume: 14, Issue:5 Pt 2

    Topics: Animals; Animals, Newborn; Behavior, Animal; Disease Models, Animal; Drug Therapy, Combination; Fetu

1994
Dihydropteridine reductase deficiency localized to the central nervous system.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:4

    Topics: Biopterins; Central Nervous System; Child; Homovanillic Acid; Humans; Hydroxyindoleacetic Acid; Male

1998
Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria.
    Neuroreport, 2000, Apr-27, Volume: 11, Issue:6

    Topics: 3,4-Dihydroxyphenylacetic Acid; Amygdala; Animals; Biogenic Monoamines; Brain; Caudate Nucleus; Dise

2000
Measurement of neurotransmitter metabolites in the cerebrospinal fluid of phenylketonuric patients under dietary treatment.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:4

    Topics: Adolescent; Adult; Aspartic Acid; Child; Dipeptides; Dopamine; Female; Homovanillic Acid; Humans; Hy

2000
Biogenic amine synthesis defect in dihydropteridine reductase deficiency.
    Science (New York, N.Y.), 1977, Nov-04, Volume: 198, Issue:4316

    Topics: 5-Hydroxytryptophan; Biogenic Amines; Carbidopa; Dopamine; Homovanillic Acid; Humans; Hydroxyindolea

1977
A disorder of biogenic amines in dihydropteridine reductase deficiency.
    Annals of neurology, 1978, Volume: 3, Issue:3

    Topics: 5-Hydroxytryptophan; Biogenic Amines; Brain; Child, Preschool; Homovanillic Acid; Humans; Hydroxyind

1978
Hyperphenylalaninaemia due to dihydropteridine reductase deficiency.
    European journal of pediatrics, 1978, Sep-08, Volume: 129, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Biopterins; Child; Child, Preschool; Female; Fibroblasts; Huma

1978
Letter: Variant forms of phenylketonuria.
    Lancet (London, England), 1976, Jun-05, Volume: 1, Issue:7971

    Topics: Biopterins; Catecholamines; Homovanillic Acid; Humans; Hydroxyindoleacetic Acid; Infant; Infant, New

1976
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; Hydr

1975
Urinary excretion of 5-hydroxyindoleacetic acid and N1-methyl-2-pyridone-5-carboxamide by normal and phenylketonuric children.
    Acta vitaminologica et enzymologica, 1975, Volume: 29, Issue:1-6

    Topics: Child; Humans; Hydroxyindoleacetic Acid; Niacinamide; Phenylketonurias; Reference Values

1975
Neurochemical and behavioral studies on the experimental phenylketonuric rats.
    The Keio journal of medicine, 1979, Volume: 28, Issue:2

    Topics: 5-Hydroxytryptophan; Animals; Brain Chemistry; Discrimination Learning; DNA; Humans; Hydroxyindoleac

1979
Atypical (mild) forms of dihydropteridine reductase deficiency: neurochemical evaluation and mutation detection.
    Pediatric research, 1992, Volume: 32, Issue:6

    Topics: Amino Acid Metabolism, Inborn Errors; Biopterins; Child, Preschool; Dihydropteridine Reductase; DNA

1992
Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents.
    European journal of pediatrics, 1985, Volume: 144, Issue:1

    Topics: Adolescent; Adult; Amino Acids; Arousal; Dopamine; Female; Homovanillic Acid; Humans; Hydroxyindolea

1985
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; R

1986
Neurotransmitter therapy and diet in malignant phenylketonuria.
    European journal of pediatrics, 1987, Volume: 146, Issue:1

    Topics: 5-Hydroxytryptophan; Carbidopa; Combined Modality Therapy; Female; Homovanillic Acid; Humans; Hydrox

1987
Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine.
    European journal of pediatrics, 1988, Volume: 148, Issue:3

    Topics: Adolescent; Adult; Female; Homovanillic Acid; Humans; Hydroxyindoleacetic Acid; Male; Neurotransmitt

1988
Prenatal diagnosis of atypical phenylketonuria.
    Journal of inherited metabolic disease, 1989, Volume: 12 Suppl 2

    Topics: Amniocentesis; Amniotic Fluid; Biomarkers; Chromatography, High Pressure Liquid; Female; Genetic Car

1989
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; Hy

1968
5-hydroxyindole levels in the blood and CSF in Down's syndrome, phenylketonuria and severe mental retardation.
    Developmental medicine and child neurology, 1972, Volume: 14, Issue:4

    Topics: Adolescent; Adult; Child; Child, Preschool; Down Syndrome; Female; Humans; Hydroxyindoleacetic Acid;

1972
Thermoregulation of phenylketonuric children.
    Pediatric research, 1974, Volume: 8, Issue:9

    Topics: Adolescent; Body Temperature Regulation; Child, Preschool; Female; Fever; Humans; Hydroxyindoleaceti

1974
[Urinary excretion of 5-hydroxyindoleacetic acid. II. In children with phenylketonuria, effects of 5-hydroxytryptophan].
    La Nouvelle presse medicale, 1973, Sep-15, Volume: 2, Issue:32

    Topics: 5-Hydroxytryptophan; Administration, Oral; Child, Preschool; Female; Humans; Hydroxyindoleacetic Aci

1973
Serotonin deficiency in experimental hyperphenylalaninemia.
    Journal of neurochemistry, 1974, Volume: 23, Issue:1

    Topics: 5-Hydroxytryptophan; Animals; Biphenyl Compounds; Brain; Carbon Radioisotopes; Carboxy-Lyases; Dopa

1974
Microanalysis with 14-C-dansyl chloride of amino acids and amines in the cerebrospinal fluid of patients with phenylketonuria. II. Determination of 5-hydroxyindole derivatives after loading with L-phenylalanine.
    Neuropadiatrie, 1974, Volume: 5, Issue:3

    Topics: 5-Hydroxytryptophan; Amines; Amino Acids; Autoradiography; Carbon Radioisotopes; Child; Child, Presc

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; Hydroxyindo

1972
Urinary 5-hydroxy-indolacetic acid in patients with atypical phenylketonuria.
    Clinica chimica acta; international journal of clinical chemistry, 1971, Volume: 34, Issue:3

    Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Hydroxyindoleacetic Acid; Male; Phenylke

1971
Tryptophan hydroxylation in phenylketonuria.
    Advances in pharmacology, 1968, Volume: 6, Issue:Pt B

    Topics: Adult; Animals; Carbon Isotopes; Chromatography, Paper; Humans; Hydroxyindoleacetic Acid; Mixed Func

1968