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Platelet serotonin concentrations in PKU patients under dietary control and tetrahydrobiopterin treatment
Author(s) -
Ormazabal A.,
Vilaseca M. A.,
PérezDueñas B.,
Lambruschini N.,
Gómez L.,
Campistol J.,
Artuch R.
Publication year - 2005
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-005-0153-3
Subject(s) - tetrahydrobiopterin , serotonin , hyperphenylalaninemia , medicine , endocrinology , platelet , phenylalanine hydroxylase , phenylalanine , chemistry , biochemistry , receptor , nitric oxide , nitric oxide synthase , amino acid
Summary Tetrahydrobiopterin (BH 4 ) supplementation has been applied in PKU treatment, resulting in successful control of blood phenylalanine (Phe) concentrations. We evaluated serotonin status in PKU patients under classical dietary treatment ( n = 40) and in a group of 11 PKU patients under BH 4 treatment, both during a 6‐month period. Platelet serotonin values were significantly lower in PKU patients under dietary treatment when compared with controls. A negative correlation was observed between plasma Phe and platelet serotonin concentrations ( r = −0.367, p = 0.017) in PKU patients. Platelet serotonin concentration increased significantly after both 1 and 6 months of BH 4 therapy when compared with baseline conditions (Wilcoxon test: p = 0.013 and p = 0.021, respectively), while no differences were observed when comparing plasma Phe concentrations at the different points. Our results indicate that PKU patients under classical treatment have decreased platelet serotonin concentrations, probably owing to continued high Phe values, while BH 4 supplementation restored platelet serotonin values.