Abstract
Phe was significantly increased (p<0,025) aad Tyr decreased (p<0,05) in platelets (pl.) from patients with PKU as compared to that of controls (2 - 14 years; HPLC-method). No correlation-was found to serum Phe/Tyr content. Amino acids calculated to pmol/108 platelets:
The in vitro uptake of 3H-Phe and 3H-Tyr by platelets showed saturation kinetics. The uptake of 3H-Tyr was not influenced by platelet Phe content. The relative release of 3H-Tyr from preincubated platelets was the same in the PKU and control group. The values of maximal 3H-Phe-and 3H-Tyr-uptake in PKU patients and controls were as followed (calculated to pmol amino acid/108 platelets):
Our data indicate that the amino acid transport of Tyr into platelets o children with PKU is reduced. The reduced concentrations of dopa/dopamine in the CSF of these patients aay be partially the result of an intracellular lack of the precursor tyrosine.
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Rolf, L., Ullrich, K., Kurlemann, G. et al. PLATELET CONTENT OF PHENYLALANINE (PHE) AND TYROSINE (TYR) SUCH AS PLATELET UPTAKE OF 3H-PHE AND 3H-TYR IN CHILDREN SUFFERING FROM PHENYLKETONURIA (PKU). Pediatr Res 22, 238 (1987). https://doi.org/10.1203/00006450-198708000-00149
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DOI: https://doi.org/10.1203/00006450-198708000-00149