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Ubiquinone-10 content in lymphocytes of phenylketonuric patients
Catrina Colomé1, Rafael Artuch, Maria A Vilaseca
1Serveis de Bioquímica, Hospital Sant Joan de Déu, Barcelona, Spain.
Objectives:
To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients.
Design And Methods:
We compared 23 patients with 25 age-matched controls. Ubiquinone-10 was analyzed by HPLC with electrochemical detection.
Results:
Ubiquinone-10 concentrations were significantly lower in patients (77-270 nmol/g of protein) compared with controls (190-550) (p < 0.001). Significantly negative correlation was observed between ubiquinone-10 and phenylalanine (r = -0.441; p < 0.05).
Conclusions:
Ubiquinone-10 concentrations are decreased in lymphocytes from phenylketonuric patients. This deficiency is associated with high plasma phenylalanine concentrations.