valine and Phenylketonurias

valine has been researched along with Phenylketonurias in 27 studies

Research

Studies (27)

TimeframeStudies, this research(%)All Research%
pre-199020 (74.07)18.7374
1990's3 (11.11)18.2507
2000's1 (3.70)29.6817
2010's2 (7.41)24.3611
2020's1 (3.70)2.80

Authors

AuthorsStudies
Cao, Z; Kong, Y; Li, T; Liu, W; Ma, Z; Rosenbaum, ER; Song, B; Wan, Z; Yue, X; Zhai, Y1
Arning, E; Bottiglieri, T; Gibson, KM; Vogel, KR; Wasek, BL1
Kohno, Y; Matsumoto, A; Nakaya, H; Sanayama, Y; Shimojo, N1
Darvish, M; Ebrahimi, SA; Ghadam, P1
TASHIAN, RE1
EFRON, ML1
Halvorsen, S; Kindt, E1
Berry, HK; Bofinger, MK; Guilfoile, MB; Hunt, MM; Phillips, PJ1
Andersen, AE; Avins, L1
Hommes, FA; Lee, JS1
Huang, SZ; Ren, ZR; Woo, SL; Zeng, YT1
Berry, HK; Brunner, RL; Hunt, MM; White, PP1
Berry, HK; Brunner, RL; Hunt, MM; Jordan, MK1
Royer, P1
Brissaud, HE1
Fekete, G1
Fateeva, EM; Nevskaia, TS1
Tomaszewski, L1
Oldendorf, WH1
Menne, F1
Rey, F; Rey, J; Sivy, M1
van der Horst, JL; Wadman, SK1
Steuer, W1
Sietti, C1
Hagge, W; Irtel von Brenndorff, A1
Blennemann, H1

Reviews

3 review(s) available for valine and Phenylketonurias

ArticleYear
AMINOACIDURIA.
    The New England journal of medicine, 1965, May-20, Volume: 272

    Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis; Glycine; Humans; Kidney; Leucine; Maple Syrup Urine Disease; Phenylketonurias; Renal Aminoacidurias; Tyrosine; Valine

1965
[Nutrition of normal and sick children].
    Pediatriia, 1973, Volume: 52, Issue:11

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Diet Therapy; Humans; Infant; Infant Nutrition Disorders; Infant Nutritional Physiological Phenomena; Nutritional Physiological Phenomena; Phenylketonurias; Protein Hydrolysates; Valine

1973
[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

Trials

1 trial(s) available for valine and Phenylketonurias

ArticleYear
Valine, isoleucine, and leucine. A new treatment for phenylketonuria.
    American journal of diseases of children (1960), 1990, Volume: 144, Issue:5

    Topics: Adolescent; Adult; Attention; Child; Double-Blind Method; Humans; Isoleucine; Language Tests; Leucine; Memory; Neuropsychological Tests; Phenylalanine; Phenylketonurias; Randomized Controlled Trials as Topic; Valine

1990

Other Studies

23 other study(ies) available for valine and Phenylketonurias

ArticleYear
Benchmark Examination of Blood Amino Acids Patterns in Phenylketonuria Neonates and Young Children on Phenylalanine-Restricted Dietary Treatment.
    Fetal and pediatric pathology, 2022, Volume: 41, Issue:3

    Topics: Arginine; Benchmarking; Child; Child, Preschool; Citrulline; Diet; Humans; Infant, Newborn; Methionine; Phenylalanine; Phenylketonurias; Proline; Tyrosine; Valine

2022
Characterization of 2-(methylamino)alkanoic acid capacity to restrict blood-brain phenylalanine transport in Pah enu2 mice: preliminary findings.
    Molecular genetics and metabolism, 2013, Volume: 110 Suppl

    Topics: Acids, Acyclic; Aminoisobutyric Acids; Animals; Blood-Brain Barrier; Brain; Disease Models, Animal; Humans; Isoleucine; Large Neutral Amino Acid-Transporter 1; Methylation; Mice; Mice, Transgenic; Molecular Targeted Therapy; Organ Specificity; Phenylalanine; Phenylketonurias; Protein Conformation; Protein Folding; Valine

2013
Phenylalanine sensitive K562-D cells for the analysis of the biochemical impact of excess amino acid.
    Scientific reports, 2014, Nov-06, Volume: 4

    Topics: Adaptor Proteins, Signal Transducing; Biological Transport; Cell Differentiation; Cell Proliferation; Fusion Regulatory Protein-1; Gene Expression Regulation; Hemin; Humans; K562 Cells; Mechanistic Target of Rapamycin Complex 1; Models, Biological; Multiprotein Complexes; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Signal Transduction; TOR Serine-Threonine Kinases; Valine

2014
Development of micellar electro kinetic chromatography for the separation and quantitation of L-valine, L-leucine, L-isoleucin and L-phenylalanine in human plasma and comparison with HPLC.
    Pakistan journal of biological sciences : PJBS, 2007, Aug-01, Volume: 10, Issue:15

    Topics: Chromatography, High Pressure Liquid; Chromatography, Micellar Electrokinetic Capillary; Humans; Isoleucine; Leucine; Maple Syrup Urine Disease; Phenylalanine; Phenylketonurias; Plasma; Reproducibility of Results; Valine

2007
Inhibition of brain glutamic acid decarboxylase by phenylalanine, valine, and leucine derivatives: a suggestion concerning the etiology of the neurological defect in phenylketonuria and branched-chain ketonuria.
    Metabolism: clinical and experimental, 1961, Volume: 10

    Topics: Amino Acids; Brain; Glutamate Decarboxylase; Humans; Intellectual Disability; Ketosis; Leucine; Lyases; Maple Syrup Urine Disease; Metabolic Diseases; Phenylalanine; Phenylketonurias; Valine

1961
The need of essential amino acids in children. An evaluation based on the intake of phenylalanine, tyrosine, leucine, isoleucine, and valine in children with phenylketonuria, tyrosine amino transferase defect, and maple syrup urine disease.
    The American journal of clinical nutrition, 1980, Volume: 33, Issue:2

    Topics: Amino Acids, Essential; Child; Child, Preschool; Dietary Proteins; Female; Humans; Isoleucine; Leucine; Male; Maple Syrup Urine Disease; Nutritional Requirements; Phenylalanine; Phenylketonurias; Protein Biosynthesis; Tyrosine; Tyrosine Transaminase; Valine

1980
Reduction of cerebrospinal fluid phenylalanine after oral administration of valine, isoleucine, and leucine.
    Pediatric research, 1982, Volume: 16, Issue:9

    Topics: Administration, Oral; Adult; Amino Acids, Branched-Chain; Child; Female; Humans; Infant; Infant, Newborn; Isoleucine; Leucine; Phenylalanine; Phenylketonurias; Tyrosine; Valine

1982
American Academy of Pediatrics. Committee on Nutrition: special diets for infants with inborn errors of amino acid metabolism.
    Pediatrics, 1976, Volume: 57, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Cysteine; Histidine; Homocystinuria; Humans; Infant; Infant, Newborn; Isoleucine; Leucine; Maple Syrup Urine Disease; Methionine; Nutritional Requirements; Phenylalanine; Phenylketonurias; Risk; Tyrosine; Valine

1976
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
The effect of plasma valine, isoleucine and leucine on the control of the flux through tyrosine- and tryptophan-hydroxylase in the brain.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:2

    Topics: Blood-Brain Barrier; Brain; Food, Formulated; Humans; Isoleucine; Leucine; Phenylketonurias; Tryptophan Hydroxylase; Tyrosine 3-Monooxygenase; Valine

1990
PAH 399 GTA (Val)----GTT(Val), a new silent mutation found in the Chinese.
    Human genetics, 1991, Volume: 86, Issue:3

    Topics: Base Sequence; China; Codon; Genes; Humans; Molecular Sequence Data; Mutation; Oligonucleotide Probes; Phenylalanine Hydroxylase; Phenylketonurias; Reference Values; Valine

1991
Preliminary support for the oral administration of valine, isoleucine and leucine for phenylketonuria.
    Developmental medicine and child neurology, 1985, Volume: 27, Issue:1

    Topics: Adolescent; Adult; Child; Cognition; Drug Therapy, Combination; Electroencephalography; Humans; Isoleucine; Learning; Leucine; Neuropsychological Tests; Phenylketonurias; Psychomotor Performance; Valine

1985
[Dietetics in hereditary enzyme deficiencies].
    La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris, 1970, Feb-26, Volume: 46, Issue:10

    Topics: Amino Acid Metabolism, Inborn Errors; Carbohydrate Metabolism, Inborn Errors; Diarrhea, Infantile; Diet Therapy; Galactosemias; Homocystinuria; Humans; Infant Nutritional Physiological Phenomena; Infant, Newborn; Isoleucine; Leucine; Maple Syrup Urine Disease; Methionine; Phenylalanine; Phenylketonurias; Tyrosine; Valine

1970
[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
[Amino acid imbalance as a pathogenetic factor in mental retardation (author's transl)].
    Klinische Wochenschrift, 1974, May-15, Volume: 52, Issue:6

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Brain Chemistry; Humans; Infant Nutritional Physiological Phenomena; Infant, Newborn; Intellectual Disability; Leucine; Methionine; Phenylalanine; Phenylketonurias; Rats; Tyrosine; Valine

1974
[The inborn errors of metabolism of amino acids].
    Postepy biochemii, 1973, Volume: 19, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Chemical Phenomena; Chemistry; Cystinuria; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; Lysine; Maple Syrup Urine Disease; Methionine; Phenylketonurias; Sarcosine; Tyrosine; Valine

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
[Rapid separation of aromatic and ramified amino acids by chromatography on ion exchange columns. Application to the surveillance of leucinosis and hyperphenylalaninemia].
    Revue europeenne d'etudes cliniques et biologiques. European journal of clinical and biological research, 1972, Volume: 17, Issue:2

    Topics: Amino Acids; Chromatography, Ion Exchange; Humans; Isoleucine; Leucine; Maple Syrup Urine Disease; Methionine; Methods; Phenylalanine; Phenylketonurias; Tyrosine; Valine

1972
A variant form of branched-chain keto aciduria.
    Acta paediatrica Scandinavica, 1971, Volume: 60, Issue:5

    Topics: Amino Acids; Child Behavior Disorders; Child, Preschool; Chromosome Aberrations; Chromosome Disorders; Diet Therapy; Dietary Proteins; Female; Heterozygote; Humans; Infant; Intellectual Disability; Isoleucine; Keto Acids; Leucine; Male; Maple Syrup Urine Disease; Methionine; Motor Skills; Pedigree; Phenylketonurias; Tyrosine; Valine

1971
[Early diagnosis of congenital metabolic diseases].
    Minerva medica, 1971, Nov-03, Volume: 62, Issue:83

    Topics: Diet Therapy; Galactosemias; Histidine; Homocystinuria; Humans; Hyperglycemia; Infant, Newborn; Leucine; Lipoproteins; Lysine; Metabolism, Inborn Errors; Phenylketonurias; Time Factors; Valine

1971
[Paper chromatography in the detection of aminoacidopathies].
    Minerva pediatrica, 1971, Dec-22, Volume: 23, Issue:51

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Chromatography, Paper; Glycine; Histidine; Humans; Maple Syrup Urine Disease; Phenylketonurias; Valine

1971
[Experiences with a new amino acid analyzer for a rapid analysis].
    Monatsschrift fur Kinderheilkunde, 1970, Volume: 118, Issue:6

    Topics: Amino Acids; Autoanalysis; Homocystine; Homocystinuria; Humans; Infant, Newborn; Infant, Premature, Diseases; Isoleucine; Leucine; Maple Syrup Urine Disease; Metabolic Diseases; Methionine; Phenylalanine; Phenylketonurias; Tyrosine; Valine

1970
[Thin-layer chromatographic diagnosis of phenylketonuria].
    Deutsche medizinische Wochenschrift (1946), 1968, Aug-16, Volume: 93, Issue:33

    Topics: Chromatography, Thin Layer; Fluorometry; Humans; Isoleucine; Leucine; Phenylalanine; Phenylketonurias; Tyrosine; Valine

1968