Plasma phenylalanine is associated with decreased serum ubiquinone-10 concentrations in phenylketonuria

Journal of Inherited Metabolic Disease

Volumn 24, Issue 3, 2001, Pages 359-366

  Artuch, R ^a   Colome C ^a   Vilaseca, M A ^a   Sierra, C ^a   Cambra, F J ^a   Lambruschini, N ^a   Campistol, J ^a  

^a UNIVERSITY OF BARCELONA   (Spain)

Author keywords

[No Author keywords available]

Indexed keywords

CHOLESTEROL; PHENYLALANINE; TYROSINE; UBIDECARENONE;

ADOLESCENT; ADULT; AMINO ACID BLOOD LEVEL; ARTICLE; CALCULATION; CHILD; CHOLESTEROL INTAKE; CLINICAL ARTICLE; CONTROLLED STUDY; CORRELATION FUNCTION; DIET THERAPY; DIETARY INTAKE; HUMAN; INFANT; PHENYLKETONURIA;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; CHOLESTEROL; CHOLESTEROL, DIETARY; HUMANS; INFANT; LINEAR MODELS; PHENYLALANINE; PHENYLKETONURIAS; TYROSINE; UBIQUINONE;

EID: 0034918034     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1010500502275     Document Type: Article

References (18)

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3. Inhibition of brain and liver 3-hydroxy-3-methylglutaryl-CoA reductase and mevalonate-5-pyrophosphate decarboxylase in experimental hyperphenylalaninemia
4. The essential functions of coenzyme Q
5. Biochemical, physiological and medical aspects of ubiquinone function
6. Activities of vitamin Q10 in animal models and a serious deficiency in patients with cancer
7. 'Hypotyrosinemia' in phenylketonuria
8. Decreased plasma ubiquinone-10 concentration in patients with mevalonate kinase deficiency
9. Coenzyme Q deficiency in mitochondria: Kinetic saturation versus physical saturation
10. The vital protonmotive role of coenzyme Q
11. Neurological outcome in adult patients with early-treated phenylketonuria
12. The hyperphenylalaninemias
13. Is there a relationship between 3-hydroxy-3-methylglutaryl coenzyme A reductase activity and forebrain pathology in the PKU mouse?
14. Mitochondrial encephalomyopathy with coenzyme Q10 deficiency
15. Ubiquinol-10 protects human low-density lipoprotein more efficiently against lipid peroxidation than does α-tocopherol
16. Large daily fluctuations in plasma tyrosine in treated patients with phenylketonuria
17. The coenzyme Q10 content of the average Danish diet
18. Exogenous coenzyme Q (CoQ) fails to increase CoQ in skeletal muscle of two patients with mitochondrial myopathies