title: Ubiquinone-10 content in lymphocytes of phenylketonuric patients.
abstract: 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.
authors.affiliation: Serveis de Bioquímica, Hospital Sant Joan de Déu, Barcelona, Spain.
authors.forename: Catrina
authors.initials: C
authors.lastname: Colomé
authors.forename: Rafael
authors.initials: R
authors.lastname: Artuch
authors.forename: Maria A
authors.initials: MA
authors.lastname: Vilaseca
authors.forename: Cristina
authors.initials: C
authors.lastname: Sierra
authors.forename: Nuria
authors.initials: N
authors.lastname: Brandi
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Cambra
authors.forename: Nilo
authors.initials: N
authors.lastname: Lambruschini
authors.forename: Jaume
authors.initials: J
authors.lastname: Campistol
mesh_terms: 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*
chemical_list: D003067:Coenzymes; D014451:Ubiquinone; D010649:Phenylalanine; C024989:coenzyme Q10