Ubiquinone-10 content in lymphocytes of phenylketonuric patients

Catrina Colomé; Rafael Artuch; Maria A. Vilaseca; Cristina Sierra; Nuria Brandi; Francisco J. Cambra; Nilo Lambruschini; Jaume Campistol

doi:10.1016/S0009-9120(02)00278-3

Ubiquinone-10 content in lymphocytes of phenylketonuric patients

Catrina Colomé, Rafael Artuch, Maria A. Vilaseca, Cristina Sierra, Nuria Brandi, Francisco J. Cambra, Nilo Lambruschini, Jaume Campistol

Producció científica: Article en revista indexada › Article › Avaluat per experts

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Objectives: To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients. Design and 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.

Idioma original	Anglès
Pàgines (de-a)	81-84
Nombre de pàgines	4
Revista	Clinical Biochemistry
Volum	35
Número	1
DOIs	https://doi.org/10.1016/S0009-9120(02)00278-3
Estat de la publicació	Publicada - 2002
Publicat externament	Sí

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@article{e037cb5f8b3948b5a052250652f9339b,

title = "Ubiquinone-10 content in lymphocytes of phenylketonuric patients",

abstract = "Objectives: To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients. Design and 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.",

keywords = "Lymphocytes, Phenylalanine, Phenylketonuria, Ubiquinone-10",

author = "Catrina Colom{\'e} and Rafael Artuch and Vilaseca, {Maria A.} and Cristina Sierra and Nuria Brandi and Cambra, {Francisco J.} and Nilo Lambruschini and Jaume Campistol",

note = "Funding Information: This work was supported by grants from FIS 00/1056 and FIS 98/0049 to 01, and from Fundaci{\'o} la Marat{\'o} TV3 de Catalunya (993710). Catrina Colom{\'e} is the recipiant of a grant from the Hospital Sant Joan de D{\'e}u.",

year = "2002",

doi = "10.1016/S0009-9120(02)00278-3",

language = "English",

volume = "35",

pages = "81--84",

journal = "Clinical Biochemistry",

issn = "0009-9120",

publisher = "Elsevier Inc.",

number = "1",

}

TY - JOUR

T1 - Ubiquinone-10 content in lymphocytes of phenylketonuric patients

AU - Colomé, Catrina

AU - Artuch, Rafael

AU - Vilaseca, Maria A.

AU - Sierra, Cristina

AU - Brandi, Nuria

AU - Cambra, Francisco J.

AU - Lambruschini, Nilo

AU - Campistol, Jaume

N1 - Funding Information: This work was supported by grants from FIS 00/1056 and FIS 98/0049 to 01, and from Fundació la Marató TV3 de Catalunya (993710). Catrina Colomé is the recipiant of a grant from the Hospital Sant Joan de Déu.

PY - 2002

Y1 - 2002

N2 - Objectives: To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients. Design and 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.

AB - Objectives: To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients. Design and 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.

KW - Lymphocytes

KW - Phenylalanine

KW - Phenylketonuria

KW - Ubiquinone-10

UR - http://www.scopus.com/inward/record.url?scp=0036218974&partnerID=8YFLogxK

U2 - 10.1016/S0009-9120(02)00278-3

DO - 10.1016/S0009-9120(02)00278-3

M3 - Article

C2 - 11937084

AN - SCOPUS:0036218974

SN - 0009-9120

VL - 35

SP - 81

EP - 84

JO - Clinical Biochemistry

JF - Clinical Biochemistry

IS - 1

ER -

Ubiquinone-10 content in lymphocytes of phenylketonuric patients

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