Page last updated: 2024-10-16

5-hydroxytryptophan and BH4 Deficiency

5-hydroxytryptophan has been researched along with BH4 Deficiency in 50 studies

5-Hydroxytryptophan: The immediate precursor in the biosynthesis of SEROTONIN from tryptophan. It is used as an antiepileptic and antidepressant.
5-hydroxytryptophan : A tryptophan derivative that is tryptophan substituted by a hydroxy group at position 5.

Research Excerpts

ExcerptRelevanceReference
"Severe 6-pyruvoyl-tetrahydrobiopterin synthase deficiency is a tetrahydrobiopterin deficiency disorder that presents in infancy with developmental delay, seizures, and abnormal movements associated with hyperphenylalaninemia usually detectable by neonatal phenylketonuria screening programs."3.736-pyruvoyl-tetrahydropterin synthase deficiency with mild hyperphenylalaninemia. ( Blau, N; Connolly, MB; Demos, MK; Hyland, K; Lillquist, Y; Makhseed, N; Vallance, HD; Waters, PJ, 2005)
"6-Pyruvoyl-tetrahydropterin synthase (PTPS) deficiency is the most important type of BH4 deficiency related to hyperphenylalaninemia."3.73Long-term follow-up of Chinese patients who received delayed treatment for 6-pyruvoyl-tetrahydropterin synthase deficiency. ( Cheng, LY; Chiu, PC; Hsiao, KJ; Lee, NC; Liu, TT; Niu, DM, 2006)
"A total of 38 cases were diagnosed as BH4 deficiency, all of them were revealed as 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency from the extremely decreased urine biopterin, normal DHPR activities and drop down of blood phenylalanine level to normal range within 4 to 8 hours after BH4 loading."3.73[Study on tetrahydrobiopterin deficiency in Northern Chinese population]. ( Chang, M; Fu, GX; He, C; Hsiao, KJ; Li, XW; Liu, TT; Shen, M; Shen, S; Wang, L; Yu, WM; Zhao, SP, 2006)
" Dietary restriction of phenylalanine and oral administration of amine precursors, L-dopa and 5-hydroxytryptophan had poor efficiency on epilepsy and psychomotor delay."3.70[Progressive convulsive encephalopathy: considering a abnormality of biopterin metabolism]. ( Dhondt, JL; Mikaeloff, Y; Pinton, F; Ponsot, G; Sevin, C, 1999)
" All patients received tetrahydrobiopterin replacement in a daily dosage between approximately 2 and 4 mg/kg."2.73Long-term follow-up of Taiwanese Chinese patients treated early for 6-pyruvoyl-tetrahydropterin synthase deficiency. ( Cheng, LY; Hsiao, KJ; Lee, NC; Liu, KM; Liu, TT; Niu, DM, 2008)
"The patients with BH4 deficiency were treated with BH4 and neurotransmitter after diagnosis."1.42Diagnosis, treatment and follow-up of patients with tetrahydrobiopterin deficiency in Shandong province, China. ( Cao, Z; Han, B; Liu, Y; Zhu, W; Zou, H, 2015)
"40 cases with BH4 deficiency were identified and all classified as PTPS deficiency between 2004 and 2012 in Shandong province, China."1.42Diagnosis, treatment and follow-up of patients with tetrahydrobiopterin deficiency in Shandong province, China. ( Cao, Z; Han, B; Liu, Y; Zhu, W; Zou, H, 2015)
"Our study emphasized that screening for BH4 deficiency should be carried out in all patients with HPA in order to minimize misdiagnosis."1.42Diagnosis, treatment and follow-up of patients with tetrahydrobiopterin deficiency in Shandong province, China. ( Cao, Z; Han, B; Liu, Y; Zhu, W; Zou, H, 2015)
"Although phenylketonuria (PKU) is the most common genetic cause of mental retardation, the cellular mechanisms underlying impaired brain function are still unclear."1.375-Hydroxytryptophan during critical postnatal period improves cognitive performances and promotes dendritic spine maturation in genetic mouse model of phenylketonuria. ( Andolina, D; Cabib, S; Conversi, D; Pascucci, T; Puglisi-Allegra, S; Trabalza, A; Ventura, R, 2011)
" This report suggests that serum prolactin levels can be a good biomarker for optimal dosage of hydroxylated precursors in long-term treatment monitoring."1.35Serum prolactin as a tool for the follow-up of treated DHPR-deficient patients. ( Concolino, D; Moricca, MT; Muzzi, G; Pascale, MG; Rapsomaniki, M; Strisciuglio, P, 2008)
"Hyperphenylalaninaemias with tetrahydrobiopterin deficiency make up about 1-3% of all hyperphenylalaninaemias."1.35Serum prolactin as a tool for the follow-up of treated DHPR-deficient patients. ( Concolino, D; Moricca, MT; Muzzi, G; Pascale, MG; Rapsomaniki, M; Strisciuglio, P, 2008)
"A total of 38 cases were diagnosed as BH4 deficiency, all of them were revealed as 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency from the extremely decreased urine biopterin, normal DHPR activities and drop down of blood phenylalanine level to normal range within 4 to 8 hours after BH4 loading."1.33[Study on tetrahydrobiopterin deficiency in Northern Chinese population]. ( Chang, M; Fu, GX; He, C; Hsiao, KJ; Li, XW; Liu, TT; Shen, M; Shen, S; Wang, L; Yu, WM; Zhao, SP, 2006)
"The differential diagnosis for BH4 deficiency should be carried out in all patients with HPA."1.33[Study on tetrahydrobiopterin deficiency in Northern Chinese population]. ( Chang, M; Fu, GX; He, C; Hsiao, KJ; Li, XW; Liu, TT; Shen, M; Shen, S; Wang, L; Yu, WM; Zhao, SP, 2006)
"The most common forms of BH4 deficiency are 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency (MIM 261640) and dihydropteridine reductase (DHPR) deficiency (MIM 261630), which require a different treatment from classical HPA."1.31Tetrahydrobiopterin-deficient hyperphenylalaninemia in the Chinese. ( Chiang, SH; Hsiao, KJ; Liu, TT; Wu, SJ, 2001)
"The high incidence of BH4 deficiency in the Taiwanese population may be explained by a founder effect, since all of the patients revealed 6-pyruvoyltetrahydropterin synthase gene mutations, and grouping N52S and P87S mutations together constituted 88."1.31Treatment and outcome of Taiwanese patients with 6-pyruvoyltetrahydropterin synthase gene mutations. ( Chiang, SC; Chien, YH; Chiu, YN; Chou, SP; Chu, SY; Huang, A; Hwu, WL; Lin, JM; Wang, TR, 2001)
"We report a child in whom dihydropteridine reductase deficiency was diagnosed prenatally because of an affected sibling and who was treated from birth with apparent good response."1.28Successful treatment of dihydropteridine reductase deficiency, with an interesting effect of 5-hydroxytryptophan deficiency on sleep patterns. ( Cotton, RG; Earl, JW; Lipson, AH; O'Halloran, M; Wilcken, B; Yu, JS, 1991)
"The mechanism of folinic acid action in DHPR deficiency may be to increase indirectly the synthesis of 5-methyltetrahydrofolate."1.27Folinic acid therapy in treatment of dihydropteridine reductase deficiency. ( Butler, IJ; Irons, M; Kaufman, S; Langlais, PJ; Levy, HL; Milstien, S; O'Flynn, ME; Stack, CV, 1987)
"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)
"A child with phenylketonuria had normal phenylalanine hydroxylase activity in vitro."1.26Atypical phenylketonuria with normal phenylalanine hydroxylase and dihydropteridine reductase activity in vitro. ( Bartholomé, K; Byrd, DJ; Kaufman, S; Milstien, S, 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 (50)

TimeframeStudies, this research(%)All Research%
pre-199031 (62.00)18.7374
1990's4 (8.00)18.2507
2000's12 (24.00)29.6817
2010's3 (6.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Han, B2
Zou, H1
Zhu, W1
Cao, Z1
Liu, Y1
Pascucci, T3
Andolina, D2
Mela, IL1
Conversi, D2
Latagliata, C1
Ventura, R3
Puglisi-Allegra, S3
Cabib, S3
Trabalza, A1
Dill, P1
Wagner, M1
Somerville, A1
Thöny, B1
Blau, N4
Weber, P1
HSIA, DY3
NISHIMURA, K1
BRENCHLEY, Y1
PERRY, TL2
TISCHLER, B1
WOOLLEY, DW2
VANDERHOEVEN, T1
JUSTICE, P1
YUWILER, A1
GELLER, E1
SLATER, GG1
LING, GM1
HANSEN, S1
MACDOUGALL, L1
Peng, SS1
Tseng, WY1
Chien, YH2
Hwu, WL2
Liu, HM1
Capistrano-Estrada, SB1
Nyhan, WL1
Demos, MK1
Waters, PJ1
Vallance, HD1
Lillquist, Y1
Makhseed, N1
Hyland, K1
Connolly, MB1
Lee, NC2
Cheng, LY2
Liu, TT4
Hsiao, KJ4
Chiu, PC1
Niu, DM2
Wang, L1
Yu, WM1
Li, XW1
He, C1
Chang, M1
Shen, M1
Zhao, SP1
Fu, GX1
Shen, S1
Sedel, F1
Ribeiro, MJ1
Remy, P1
Saudubray, JM1
Agid, Y1
Liu, KM1
Concolino, D1
Muzzi, G1
Rapsomaniki, M1
Moricca, MT1
Pascale, MG1
Strisciuglio, P1
Beck, B1
Brandt, NJ1
Christensen, E1
Niederwieser, A3
Pedersen, PS1
Endres, W1
Curtius, HC1
Wang, M1
Ohrt, B1
Schaub, J1
Salomon, F1
Fagioli, I1
Rey, F1
Salzarulo, P1
Piel, N1
Lane, JD1
Hüther, G1
Neuhoff, V4
Tsukada, Y2
Takamatsu, K1
Goldstein, DS1
Hahn, SH1
Holmes, C1
Tifft, C1
Harvey-White, J1
Milstien, S4
Kaufman, S5
Dhondt, JL2
Mikaeloff, Y1
Pinton, F1
Sevin, C1
Ponsot, G1
Chiang, SH1
Wu, SJ1
Chiang, SC1
Huang, A1
Lin, JM1
Chiu, YN1
Chou, SP1
Chu, SY1
Wang, TR1
Koslow, SH2
Butler, IJ3
Krumholz, A1
Holtzman, NA1
Bartholomé, K2
Byrd, DJ2
Airaksinen, MM1
Turakka, H1
Marvola, M1
MacDonald, EJ1
Luchins, D1
Brewster, TG1
Moskowitz, MA1
Breslow, JL1
Abroms, IF1
Kohsaka, S1
Schafer, EW1
McKean, CM1
Lipson, AH1
Earl, JW1
Wilcken, B1
Yu, JS1
O'Halloran, M1
Cotton, RG1
Ponzone, A1
Guardamagna, O1
Ferraris, S1
Biasetti, S1
Bracco, G1
Irons, M1
Levy, HL1
O'Flynn, ME1
Stack, CV1
Langlais, PJ1
Cohen, BE1
Szeinberg, A1
Quint, J1
Normand, M1
Blonder, J1
Peled, I1
Pare, CM1
Costil, J1
Galli, J1
Richardet, JM1
Brissaud, HE1
Quentin, CD3
Behbehani, AW2
Schulte, FJ2
Loo, YH1
Behbehani, A1
Prinz, H1
Van der Hoeven, T1

Reviews

3 reviews available for 5-hydroxytryptophan and BH4 Deficiency

ArticleYear
[Correlation between monoamine content in the brain and discriminative learning ability (author's transl)].
    Tanpakushitsu kakusan koso. Protein, nucleic acid, enzyme, 1981, Volume: 26, Issue:11

    Topics: 5-Hydroxytryptophan; Animals; Biogenic Amines; Brain; Discrimination Learning; Humans; Macaca mulatt

1981
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

1976
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

1967

Trials

3 trials available for 5-hydroxytryptophan and BH4 Deficiency

ArticleYear
Long-term follow-up of Taiwanese Chinese patients treated early for 6-pyruvoyl-tetrahydropterin synthase deficiency.
    Archives of neurology, 2008, Volume: 65, Issue:3

    Topics: 5-Hydroxytryptophan; Adolescent; Adult; Asian People; Child; Child, Preschool; Female; Humans; Infan

2008
Atypical phenylketonuria due to biopterin deficiency. Early treatment with tetrahydrobiopterin and neurotransmitter precursors, trials of monotherapy.
    Helvetica paediatrica acta, 1982, Volume: 37, Issue:5

    Topics: 5-Hydroxytryptophan; Biopterins; Carbidopa; Clinical Trials as Topic; Humans; Infant; Levodopa; Male

1982
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

1976

Other Studies

45 other studies available for 5-hydroxytryptophan and BH4 Deficiency

ArticleYear
Diagnosis, treatment and follow-up of patients with tetrahydrobiopterin deficiency in Shandong province, China.
    Brain & development, 2015, Volume: 37, Issue:6

    Topics: 5-Hydroxytryptophan; Biopterins; Child, Preschool; China; Follow-Up Studies; Genotype; Humans; Infan

2015
5-Hydroxytryptophan rescues serotonin response to stress in prefrontal cortex of hyperphenylalaninaemic mice.
    The international journal of neuropsychopharmacology, 2009, Volume: 12, Issue:8

    Topics: 5-Hydroxytryptophan; Analysis of Variance; Animals; Antidepressive Agents, Second-Generation; Diet,

2009
5-Hydroxytryptophan during critical postnatal period improves cognitive performances and promotes dendritic spine maturation in genetic mouse model of phenylketonuria.
    The international journal of neuropsychopharmacology, 2011, Volume: 14, Issue:4

    Topics: 5-Hydroxytryptophan; Animals; Behavior, Animal; Cognition; Cognition Disorders; Critical Period, Psy

2011
Child neurology: paroxysmal stiffening, upward gaze, and hypotonia: hallmarks of sepiapterin reductase deficiency.
    Neurology, 2012, Jan-31, Volume: 78, Issue:5

    Topics: 5-Hydroxytryptophan; Alcohol Oxidoreductases; Behavior; Benserazide; Consanguinity; Developmental Di

2012
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
MECHANISMS FOR THE DECREASE OF BRAIN SEROTONIN.
    Nature, 1963, Nov-09, Volume: 200

    Topics: 5-Hydroxytryptophan; Animals; Animals, Newborn; Blood Chemical Analysis; Brain; Carboxy-Lyases; Diet

1963
5-HYDROXYTRYPTOPHAN ADMINISTRATION IN PHENYLKETONURIA.
    American journal of diseases of children (1960), 1964, Volume: 107

    Topics: 5-Hydroxytryptophan; Biomedical Research; Brain; Child; Diet; Electroencephalography; Humans; Indole

1964
PREVENTION OF A MENTAL DEFECT OF PHENYLKETONURIA WITH SEROTONIN CONGENERS SUCH AS MELATONIN OR HYDROXYTRYPTOPHAN.
    Science (New York, N.Y.), 1964, Jun-26, Volume: 144, Issue:3626

    Topics: 5-Hydroxytryptophan; Animals; Biomedical Research; Indoles; Learning; Melatonin; Mice; Pharmacology;

1964
STUDIES ON INHIBITION OF BRAIN 5-HYDROXYTRYPTOPHAN DECARBOXYLASE BY PHENYLALANINE METABOLITES.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1965, Volume: 118

    Topics: 5-Hydroxytryptophan; Amino Acids; Animals; Antimetabolites; Aromatic-L-Amino-Acid Decarboxylases; Br

1965
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; C

1965
UNIMPAIRED LEARNING ABILITY OF RATS MADE ARTIFICIALLY PHENYLKETONURIC DURING FETAL OR NEONATAL LIFE.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1965, Volume: 119

    Topics: 5-Hydroxytryptophan; Animals; Animals, Newborn; Brain Chemistry; Diet; Discrimination Learning; Fetu

1965
Diffusion tensor images in children with early-treated, chronic, malignant phenylketonuric: correlation with intelligence assessment.
    AJNR. American journal of neuroradiology, 2004, Volume: 25, Issue:9

    Topics: 5-Hydroxytryptophan; Axons; Biopterins; Brain Damage, Chronic; Cerebral Cortex; Child; Child, Presch

2004
6-pyruvoyl tetrahydropterin synthase deficiency: a case report.
    The Southeast Asian journal of tropical medicine and public health, 2003, Volume: 34 Suppl 3

    Topics: 5-Hydroxytryptophan; Biopterins; Dihydroxyphenylalanine; Female; Humans; Infant, Newborn; Neonatal S

2003
6-pyruvoyl-tetrahydropterin synthase deficiency with mild hyperphenylalaninemia.
    Annals of neurology, 2005, Volume: 58, Issue:1

    Topics: 5-Hydroxytryptophan; Biopterins; Carbidopa; Cerebral Palsy; Child; Developmental Disabilities; Diagn

2005
Long-term follow-up of Chinese patients who received delayed treatment for 6-pyruvoyl-tetrahydropterin synthase deficiency.
    Molecular genetics and metabolism, 2006, Volume: 87, Issue:2

    Topics: 5-Hydroxytryptophan; Adolescent; Adult; Asian People; Biopterins; Child; Child, Preschool; China; Fe

2006
[Study on tetrahydrobiopterin deficiency in Northern Chinese population].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2006, Volume: 23, Issue:3

    Topics: 5-Hydroxytryptophan; Asian People; Biopterins; Child, Preschool; China; Dihydropteridine Reductase;

2006
Dihydropteridine reductase deficiency: levodopa's long-term effectiveness without dyskinesia.
    Neurology, 2006, Dec-26, Volume: 67, Issue:12

    Topics: 5-Hydroxytryptophan; Adult; Antidepressive Agents, Second-Generation; Dopamine Agents; Drug Combinat

2006
Serum prolactin as a tool for the follow-up of treated DHPR-deficient patients.
    Journal of inherited metabolic disease, 2008, Volume: 31 Suppl 2

    Topics: 5-Hydroxytryptophan; Aromatic Amino Acid Decarboxylase Inhibitors; Biomarkers; Carbidopa; Child, Pre

2008
Diagnostic and therapeutic aspects of dihydrobiopterin deficiency.
    Acta paediatrica Scandinavica, 1983, Volume: 72, Issue:3

    Topics: 5-Hydroxytryptophan; Biopterins; Carbidopa; Chromatography, High Pressure Liquid; Dihydroxyphenylala

1983
Sleep patterns in the "new variant" phenylketonuria.
    Neuropediatrics, 1981, Volume: 12, Issue:3

    Topics: 5-Hydroxytryptophan; Dopamine; Humans; Infant; NADH, NADPH Oxidoreductases; Phenylketonurias; Sleep

1981
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; Ca

1982
Monoaminergic effects of folinic acid, L-DOPA, and 5-hydroxytryptophan in dihydropteridine reductase deficiency.
    Journal of neurochemistry, 1995, Volume: 64, Issue:6

    Topics: 5-Hydroxytryptophan; Biogenic Monoamines; Humans; Infant; Leucovorin; Levodopa; Male; Phenylketonuri

1995
Tetrahydrobiopterin deficiency and an international database of patients.
    Advances in experimental medicine and biology, 1993, Volume: 338

    Topics: 5-Hydroxytryptophan; Alcohol Oxidoreductases; Biomarkers; Biopterins; Carbidopa; Child; Dihydropteri

1993
[Progressive convulsive encephalopathy: considering a abnormality of biopterin metabolism].
    Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 1999, Volume: 6, Issue:7

    Topics: 5-Hydroxytryptophan; Algeria; Anticonvulsants; Biopterins; Brain; Child, Preschool; Consanguinity; D

1999
Tetrahydrobiopterin-deficient hyperphenylalaninemia in the Chinese.
    Clinica chimica acta; international journal of clinical chemistry, 2001, Volume: 313, Issue:1-2

    Topics: 5-Hydroxytryptophan; Biopterins; Diagnosis, Differential; Dihydropteridine Reductase; Female; Geneti

2001
Treatment and outcome of Taiwanese patients with 6-pyruvoyltetrahydropterin synthase gene mutations.
    Journal of inherited metabolic disease, 2001, Volume: 24, Issue:8

    Topics: 5-Hydroxytryptophan; Base Sequence; Biopterins; DNA Mutational Analysis; Founder Effect; Humans; Inf

2001
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
Letter: L-dopa and 5-hydroxytryptophan therapy in phenylketonuria with normal phenylalanine-hydroxylase activity.
    Lancet (London, England), 1975, Nov-22, Volume: 2, Issue:7943

    Topics: 5-Hydroxytryptophan; Carbidopa; Drug Therapy, Combination; Humans; Infant; Levodopa; Phenylalanine H

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; Hydr

1975
Atypical phenylketonuria with normal phenylalanine hydroxylase and dihydropteridine reductase activity in vitro.
    Pediatrics, 1977, Volume: 59, Issue:5

    Topics: 5-Hydroxytryptophan; Dihydropteridine Reductase; Humans; Infant, Newborn; Levodopa; Liver; Male; NAD

1977
Dihydropteridine reductase deficiency associated with severe neurologic disease and mild hyperphenylalaninemia.
    Pediatrics, 1979, Volume: 63, Issue:1

    Topics: 5-Hydroxytryptophan; Ascorbic Acid; Consanguinity; Dopamine; Humans; Infant; Levodopa; Liver; Male;

1979
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
Evidence that monoamines influence human evoked potentials.
    Brain research, 1975, Nov-28, Volume: 99, Issue:1

    Topics: 5-Hydroxytryptophan; Adolescent; Biogenic Amines; Dihydroxyphenylalanine; Evoked Potentials; Female;

1975
Successful treatment of dihydropteridine reductase deficiency, with an interesting effect of 5-hydroxytryptophan deficiency on sleep patterns.
    Journal of inherited metabolic disease, 1991, Volume: 14, Issue:1

    Topics: 5-Hydroxytryptophan; Female; Humans; Levodopa; Male; Phenylketonurias; Pregnancy; Prenatal Diagnosis

1991
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
Folinic acid therapy in treatment of dihydropteridine reductase deficiency.
    The Journal of pediatrics, 1987, Volume: 110, Issue:1

    Topics: 5-Hydroxytryptophan; Brain; Carbidopa; Drug Therapy, Combination; Female; Folic Acid; Humans; Infant

1987
Malignant phenylketonuria due to defective synthesis of dihydrobiopterin.
    Israel journal of medical sciences, 1985, Volume: 21, Issue:6

    Topics: 5-Hydroxytryptophan; Biopterins; Carbidopa; Drug Combinations; Female; Humans; Infant, Newborn; Levo

1985
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
[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
Microanalysis with 14C-dansyl chloride of amino acids and amines in the cerebrospinal fluid of patients with phenylketonuria. I. Analysis in untreated phenylketonuria.
    Neuropadiatrie, 1974, Volume: 5, Issue:2

    Topics: 5-Hydroxytryptophan; Amines; Amino Acids; Autoradiography; Carbon Radioisotopes; Chlorides; Chromato

1974
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
Identification of 5-hydroxytryptophan by dansylation in the cerebrospinal fluid of children with phenylketonuria.
    Hoppe-Seyler's Zeitschrift fur physiologische Chemie, 1974, Volume: 355, Issue:7

    Topics: 5-Hydroxytryptophan; Child; Chromatography, Thin Layer; Dansyl Compounds; Humans; Phenylketonurias;

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
Serotonin deficiency in infancy as a cause of a mental defect in experimental phenylketonuria.
    International journal of neuropsychiatry, 1965, Volume: 1, Issue:6

    Topics: 5-Hydroxytryptophan; Animals; Animals, Newborn; Chlorpromazine; Electroshock; Female; Humans; In Vit

1965