isoleucine has been researched along with BH4 Deficiency in 19 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 14 (73.68) | 18.7374 |
| 1990's | 3 (15.79) | 18.2507 |
| 2000's | 1 (5.26) | 29.6817 |
| 2010's | 1 (5.26) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Arning, E; Bottiglieri, T; Gibson, KM; Vogel, KR; Wasek, BL | 1 |
| Darvish, M; Ebrahimi, SA; Ghadam, P | 1 |
| GELLER, E; SLATER, GG; YUWILER, A | 1 |
| Halvorsen, S; Kindt, E | 1 |
| Berry, HK; Bofinger, MK; Guilfoile, MB; Hunt, MM; Phillips, PJ | 1 |
| Berry, HK; Brunner, RL; Butcher, RE; McLean, MS; Vorhees, CV | 1 |
| Andersen, AE; Avins, L | 1 |
| Miller, JB; Qu, Y; Shapira, E; Slocum, RH | 1 |
| Hommes, FA; Lee, JS | 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 |
| Oldendorf, WH | 1 |
| Rey, F; Rey, J; Sivy, M | 1 |
| van der Horst, JL; Wadman, SK | 1 |
| Hagge, W; Irtel von Brenndorff, A | 1 |
| Blennemann, H | 1 |
1 trial(s) available for isoleucine 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 |
18 other study(ies) available for isoleucine and BH4 Deficiency
| Article | Year |
|---|---|
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 |
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 |
DIETARY ISOLEUCINE INHIBITION OF LIVER TRYPTOPHAN HYDROXYLASE.
Topics: Brain; Carboxy-Lyases; Diet; Isoleucine; Liver; Maple Syrup Urine Disease; Mixed Function Oxygenases; Phenylketonurias; Rats; Research; Tryptophan Hydroxylase | 1964 |
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 |
Beneficial effect of isoleucine on fetal brain development in induced phenylketonuria.
Topics: Amino Acids; Animals; Body Weight; Brain; Disease Models, Animal; Female; Humans; Isoleucine; Organ Size; Phenylalanine; Phenylketonurias; Pregnancy; Rats | 1978 |
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 |
Rapid automated quantitation of isoleucine, leucine, tyrosine and phenylalanine from dried blood filter paper specimens.
Topics: Amino Acid Metabolism, Inborn Errors; Autoanalysis; Chromatography, High Pressure Liquid; Humans; Infant, Newborn; Isoleucine; Leucine; Maple Syrup Urine Disease; Neonatal Screening; Paper; Phenylalanine; Phenylketonurias; Tyrosine | 1991 |
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 |
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 |
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 |
[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 |