valine has been researched along with BH4 Deficiency in 27 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 20 (74.07) | 18.7374 |
| 1990's | 3 (11.11) | 18.2507 |
| 2000's | 1 (3.70) | 29.6817 |
| 2010's | 2 (7.41) | 24.3611 |
| 2020's | 1 (3.70) | 2.80 |
| Authors | Studies |
|---|---|
| Cao, Z; Kong, Y; Li, T; Liu, W; Ma, Z; Rosenbaum, ER; Song, B; Wan, Z; Yue, X; Zhai, Y | 1 |
| Arning, E; Bottiglieri, T; Gibson, KM; Vogel, KR; Wasek, BL | 1 |
| Kohno, Y; Matsumoto, A; Nakaya, H; Sanayama, Y; Shimojo, N | 1 |
| Darvish, M; Ebrahimi, SA; Ghadam, P | 1 |
| TASHIAN, RE | 1 |
| EFRON, ML | 1 |
| Halvorsen, S; Kindt, E | 1 |
| Berry, HK; Bofinger, MK; Guilfoile, MB; Hunt, MM; Phillips, PJ | 1 |
| Andersen, AE; Avins, L | 1 |
| Hommes, FA; Lee, JS | 1 |
| Huang, SZ; Ren, ZR; Woo, SL; Zeng, YT | 1 |
| Berry, HK; Brunner, RL; Hunt, MM; White, PP | 1 |
| Berry, HK; Brunner, RL; Hunt, MM; Jordan, MK | 1 |
| Royer, P | 1 |
| Brissaud, HE | 1 |
| Fekete, G | 1 |
| Fateeva, EM; Nevskaia, TS | 1 |
| Tomaszewski, L | 1 |
| Oldendorf, WH | 1 |
| Menne, F | 1 |
| Rey, F; Rey, J; Sivy, M | 1 |
| van der Horst, JL; Wadman, SK | 1 |
| Steuer, W | 1 |
| Sietti, C | 1 |
| Hagge, W; Irtel von Brenndorff, A | 1 |
| Blennemann, H | 1 |
3 review(s) available for valine and BH4 Deficiency
| Article | Year |
|---|---|
AMINOACIDURIA.
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].
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].
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 |
1 trial(s) available for valine and BH4 Deficiency
| Article | Year |
|---|---|
Valine, isoleucine, and leucine. A new treatment for phenylketonuria.
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 |
23 other study(ies) available for valine and BH4 Deficiency
| Article | Year |
|---|---|
Benchmark Examination of Blood Amino Acids Patterns in Phenylketonuria Neonates and Young Children on Phenylalanine-Restricted Dietary Treatment.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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].
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].
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)].
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].
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.
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].
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.
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].
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].
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].
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].
Topics: Chromatography, Thin Layer; Fluorometry; Humans; Isoleucine; Leucine; Phenylalanine; Phenylketonurias; Tyrosine; Valine | 1968 |