Document Detail


Monoaminergic effects of folinic acid, L-DOPA, and 5-hydroxytryptophan in dihydropteridine reductase deficiency.
MedLine Citation:
PMID:  7760062     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Plasma and CSF concentrations of endogenous L-DOPA, catecholamines, and metabolites of monoamines were assayed in a patient with atypical phenylketonuria due to absent dihydropteridine reductase (DHPR), before and during treatment with folinic acid, Sinemet, and 5-hydroxytryptophan. The patient had low but detectable levels of L-DOPA, 3,4-dihydroxyphenylacetic acid (DOPAC), and 3,4-dihydroxyphenylglycol (DHPG) in plasma and low but detectable levels of these compounds and of homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) in CSF, with approximately normal plasma and CSF levels of norepinephrine [noradrenaline (NA)]. Folinic acid treatment approximately doubled plasma levels of L-DOPA, NA, DOPAC, and DHPG, compared with values during dietary phenylalanine restriction alone. Detection of L-DOPA, catecholamines, and monoamine metabolites in this patient indicates that monoamine synthesis in humans does not absolutely require DHPR. The results are consistent with the existence of an alternative biochemical pathway, with folinic acid treatment augmenting activity along this pathway. Low plasma levels of L-DOPA, DOPAC, and DHPG may reflect decreased catecholamine synthesis and turnover in sympathetic nerves, with compensatory increases in exocytotic release normalizing plasma NA levels.
Authors:
D S Goldstein; S H Hahn; C Holmes; C Tifft; J Harvey-White; S Milstien; S Kaufman
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of neurochemistry     Volume:  64     ISSN:  0022-3042     ISO Abbreviation:  J. Neurochem.     Publication Date:  1995 Jun 
Date Detail:
Created Date:  1995-06-28     Completed Date:  1995-06-28     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  2985190R     Medline TA:  J Neurochem     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2810-3     Citation Subset:  IM    
Affiliation:
Clinical Neuroscience Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD 20892, USA.
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MeSH Terms
Descriptor/Qualifier:
5-Hydroxytryptophan / therapeutic use*
Biogenic Monoamines / metabolism*
Humans
Infant
Leucovorin / therapeutic use*
Levodopa / therapeutic use*
Male
Phenylketonurias*
Chemical
Reg. No./Substance:
0/Biogenic Monoamines; 0/Levodopa; 56-69-9/5-Hydroxytryptophan; 58-05-9/Leucovorin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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