Decreased serum ubiquinone-10 concentrations in phenylketonuria.
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.


METHODS
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 <8 y and P < 0.00005 for patients aged 8-33 y). Moreover, 5 of 18 PKU patients (28%) in the younger age group and 10 of 23 (43%) in the older age group had serum ubiquinone-10 concentrations below the reference interval.


CONCLUSIONS
Serum ubiquinone-10 deficiency appears to be related to the restricted diet of PKU patients. Because serum ubiquinone-10 plays a major antioxidant role in the protection of circulating lipoproteins, the correction of ubiquinone-10 concentrations should be considered in PKU patients. Further investigation seems advisable to elucidate whether the deficiency in serum ubiquinone-10 status is clinically significant.
Artuch
R
R
Servei de Bioquímica, Servei de Pediatría i Neuropediatría, Unitat Integrada, Hospital Sant Joan de Déu/Hospital Clinic, Universitat de Barcelona, Spain.
Vilaseca
M A
MA
Moreno
J
J
Lambruschini
N
N
Cambra
F J
FJ
Campistol
J
J
D000293:Adolescent; D000328:Adult; D000931:Antidotes / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood*; D002851:Chromatography, High Pressure Liquid; D002852:Chromatography, Ion Exchange; D004032:Diet; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000097:blood*; D012016:Reference Values; D012189:Retrospective Studies; D014443:Tyrosine / Q000097:blood*; D014451:Ubiquinone / Q000097:blood*
D000931:Antidotes; D014451:Ubiquinone; D014443:Tyrosine; D010649:Phenylalanine; D002784:Cholesterol; C025204:Ubiquinone Q2