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dihydroxyphenylalanine and Lesch-Nyhan Syndrome

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)

Research Excerpts

ExcerptRelevanceReference
"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."1.29Presynaptic 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)

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19901 (50.00)18.7374
1990's1 (50.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Ernst, M1
Zametkin, AJ1
Matochik, JA1
Pascualvaca, D1
Jons, PH1
Hardy, K1
Hankerson, JG1
Doudet, DJ1
Cohen, RM1
Mizuno, TI1
Yugari, Y1

Trials

1 trial available for dihydroxyphenylalanine and Lesch-Nyhan Syndrome

ArticleYear
Letter: Self mutilation in Lesch-Nyhan syndrome.
    Lancet (London, England), 1974, Apr-20, Volume: 1, Issue:7860

    Topics: 5-Hydroxytryptophan; Administration, Oral; Child; Dihydroxyphenylalanine; Humans; Hydroxyindoleaceti

1974

Other Studies

1 other study available for dihydroxyphenylalanine and Lesch-Nyhan Syndrome

ArticleYear
Presynaptic dopaminergic deficits in Lesch-Nyhan disease.
    The New England journal of medicine, 1996, Jun-13, Volume: 334, Issue:24

    Topics: Adolescent; Adult; Analysis of Variance; Brain; Brain Chemistry; Caudate Nucleus; Cerebral Cortex; C

1996