Ubiquinone-10 content in lymphocytes of phenylketonuric patients.
OBJECTIVE
To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients.


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.
Colomé
Catrina
C
Serveis de Bioquímica, Hospital Sant Joan de Déu, Barcelona, Spain.
Artuch
Rafael
R
Vilaseca
Maria A
MA
Sierra
Cristina
C
Brandi
Nuria
N
Cambra
Francisco J
FJ
Lambruschini
Nilo
N
Campistol
Jaume
J
D000293:Adolescent; D000328:Adult; D002648:Child; D002851:Chromatography, High Pressure Liquid; D003067:Coenzymes; D004032:Diet; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000378:metabolism*; D008297:Male; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000097:blood*; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood*
D003067:Coenzymes; D014451:Ubiquinone; D010649:Phenylalanine; C024989:coenzyme Q10