Decreased serum ubiquinone-10 concentrations in phenylketonuria

Authors :: Francisco José Cambra
Jaume Campistol
N. Lambruschini
Rafael Artuch
Juan J. Moreno
M-Antònia Vilaseca
Source :: AMERICAN JOURNAL OF CLINICAL NUTRITION, r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu, Fundació Sant Joan de Déu, r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu, instname
Publication Year :: 1999
Publisher :: Elsevier BV, 1999.
Abstract: Background: Ubiquinone-10 is a lipid with important metabolic functions that may be decreased in phenylketonuria (PKU) because patients with PKU consume diets restricted in natural proteins. Objective: We studied serum ubiquinone-10 concentrations in PKU patients. Design: This was a retrospective, transversal study in which we compared serum ubiquinone-10, plasma cholesterol, plasma tyrosine, and plasma phenylalanine concentrations in 43 PKU patients with concentrations in a reference population (n = 102). Serum ubiquinone-10 concentrations were analyzed by HPLC with ultraviolet detection. Plasma tyrosine and phenylalanine were measured by ion-exchange chromatography. Results: Serum ubiquinone-10 concentrations in PKU patients were significantly lower than in the reference population (P < 0.01 for patients aged 1 mo to