dihydroxyphenylalanine has been researched along with Lesch-Nyhan Syndrome in 2 studies
Dihydroxyphenylalanine: A beta-hydroxylated derivative of phenylalanine. The D-form of dihydroxyphenylalanine has less physiologic activity than the L-form and is commonly used experimentally to determine whether the pharmacological effects of LEVODOPA are stereospecific.
dopa : A hydroxyphenylalanine carrying hydroxy substituents at positions 3 and 4 of the benzene ring.
Lesch-Nyhan Syndrome: An inherited disorder transmitted as a sex-linked trait and caused by a deficiency of an enzyme of purine metabolism; HYPOXANTHINE PHOSPHORIBOSYLTRANSFERASE. Affected individuals are normal in the first year of life and then develop psychomotor retardation, extrapyramidal movement disorders, progressive spasticity, and seizures. Self-destructive behaviors such as biting of fingers and lips are seen frequently. Intellectual impairment may also occur but is typically not severe. Elevation of uric acid in the serum leads to the development of renal calculi and gouty arthritis. (Menkes, Textbook of Child Neurology, 5th ed, pp127)
Excerpt | Relevance | Reference |
---|---|---|
"The fluorodopa F 18 ratio was significantly lower in the putamen (31 percent of control values), caudate nucleus (39 percent), frontal cortex (44 percent), and ventral tegmental complex (substantia nigra and ventral tegmentum; 57 percent) in the patients with Lesch-Nyhan disease than in the controls." | 1.29 | Presynaptic dopaminergic deficits in Lesch-Nyhan disease. ( Cohen, RM; Doudet, DJ; Ernst, M; Hankerson, JG; Hardy, K; Jons, PH; Matochik, JA; Pascualvaca, D; Zametkin, AJ, 1996) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 1 (50.00) | 18.7374 |
1990's | 1 (50.00) | 18.2507 |
2000's | 0 (0.00) | 29.6817 |
2010's | 0 (0.00) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
---|---|
Ernst, M | 1 |
Zametkin, AJ | 1 |
Matochik, JA | 1 |
Pascualvaca, D | 1 |
Jons, PH | 1 |
Hardy, K | 1 |
Hankerson, JG | 1 |
Doudet, DJ | 1 |
Cohen, RM | 1 |
Mizuno, TI | 1 |
Yugari, Y | 1 |
1 trial available for dihydroxyphenylalanine and Lesch-Nyhan Syndrome
Article | Year |
---|---|
Letter: Self mutilation in Lesch-Nyhan syndrome.
Topics: 5-Hydroxytryptophan; Administration, Oral; Child; Dihydroxyphenylalanine; Humans; Hydroxyindoleaceti | 1974 |
1 other study available for dihydroxyphenylalanine and Lesch-Nyhan Syndrome
Article | Year |
---|---|
Presynaptic dopaminergic deficits in Lesch-Nyhan disease.
Topics: Adolescent; Adult; Analysis of Variance; Brain; Brain Chemistry; Caudate Nucleus; Cerebral Cortex; C | 1996 |