• Clinical biochemistry · Feb 2002

    Comparative Study

    Ubiquinone-10 content in lymphocytes of phenylketonuric patients.

    • Catrina Colomé, Rafael Artuch, Maria A Vilaseca, Cristina Sierra, Nuria Brandi, Francisco J Cambra, Nilo Lambruschini, and Jaume Campistol.
    • Serveis de Bioquímica, Hospital Sant Joan de Déu, Barcelona, Spain.
    • Clin. Biochem. 2002 Feb 1; 35 (1): 81-4.

    ObjectivesTo investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients.Design And MethodsWe compared 23 patients with 25 age-matched controls. Ubiquinone-10 was analyzed by HPLC with electrochemical detection.ResultsUbiquinone-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).ConclusionsUbiquinone-10 concentrations are decreased in lymphocytes from phenylketonuric patients. This deficiency is associated with high plasma phenylalanine concentrations.

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