Lipophilic antioxidants in patients with phenylketonuria

Colomé, Catrina; Artuch, Rafael; Vilaseca, M A; Sierra, Cristina; Brandi, Nuria; Lambruschini, Nilo; Cambra, Francisco José; Campistol, Jaume

doi:10.1093/ajcn/77.1.185

Cited by 59 publications

(71 citation statements)

References 20 publications

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“…Both catalase and glucose-6-phosphate dehydrogenase were significantly increased in the red blood cells of PKU mice, also indicating oxidative stress. Colome et al (19) observed in patients with PKU similar findings that showed increased malondialdehyde along with other biochemical markers of oxidative stress and concluded that plasma lipid peroxidation is increased in these patients. Hagen et al (20) demonstrated in an experimentally induced rat model of hyperphenylalanemia that excessive Phe stimulates oxidative stress in the brain.…”

Section: Discussionmentioning

confidence: 59%

Pathologic and Immunohistochemical Findings in Hypothalamic and Mesencephalic Regions in the Pahenu2 Mouse Model for Phenylketonuria

2005

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Section: Discussionmentioning

confidence: 59%

Pathologic and Immunohistochemical Findings in Hypothalamic and Mesencephalic Regions in the Pahenu2 Mouse Model for Phenylketonuria

2005

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“…An increase of malondialdehyde, which is a marker of lipid peroxidation, was observed in plasma of PKU patients at diagnosis and during dietary treatment (Colomé et al 2003;Gassió et al 2008;Sitta et al 2006Sitta et al , 2009bSirtori et al 2005). In addition, increased lipid and protein oxidative damage was also verified in plasma from affected patients with late diagnosis as compared to early diagnosed PKU patients (Sitta et al 2009c).…”

Section: Oxidative Stress In Pku Patientsmentioning

confidence: 99%

“…Ubiquinone-10 or coenzyme Q 10 (Q 10 ), a substance with antioxidant action, was also found to be decreased in serum from PKU patients (Artuch et al 1999;Gassió et al 2008;Colomé et al 2003;Artuch et al 2004). Q 10 is a lipophilic antioxidant that prevents lipid peroxidation in blood and tissues by limiting the formation of lipid peroxyl radicals and by reducing other natural antioxidant compounds, specifically the conversion of tocopheroxyl to tocopherol (Colomé et al 2003).…”

Section: Oxidative Stress In Pku Patientsmentioning

confidence: 99%

Oxidative Stress in Phenylketonuria: What is the Evidence?

et al. 2011

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Phenylketonuria (PKU) is an inborn error of amino acid metabolism caused by severe deficiency of phenylalanine hydroxylase activity, leading to the accumulation of phenylalanine and its metabolites in blood and tissues of affected patients. Phenylketonuric patients present as the major clinical feature mental retardation, whose pathomechanisms are poorly understood. In recent years, mounting evidence has emerged indicating that oxidative stress is possibly involved in the pathology of PKU. This article addresses some of the recent developments obtained from animal studies and from phenylketonuric patients indicating that oxidative stress may represent an important element in the pathophysiology of PKU. Several studies have shown that enzymatic and non-enzymatic antioxidant defenses are decreased in plasma and erythrocytes of PKU patients, which may be due to an increased free radical generation or secondary to the deprivation of micronutrients which are essential for these defenses. Indeed, markers of lipid, protein, and DNA oxidative damage have been reported in PKU patients, implying that reactive species production is increased in this disorder. A considerable set of data from in vitro and in vivo animal studies have shown that phenylalanine and/or its metabolites elicit reactive species in brain rodent. These findings point to a disruption of pro-oxidant/antioxidant balance in PKU. Considering that the brain is particularly vulnerable to oxidative attack, it is presumed that the administration of appropriate antioxidants as adjuvant agents, in addition to the usual treatment based on restricted diets or supplementation of tetrahydrobiopterin, may represent another step in the prevention of the neurological damage in PKU.

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“…phenylketonuric patients), with no dietary introduction of CoQ 10 or precursors [14,15], or to deficiencies involving the biosynthetic pathway or the mechanism of distribution [16]; therefore, tissue metabolic requests and time of consumption of tissue CoQ 10 have to be evaluated as well [17].…”

Section: Introductionmentioning

confidence: 99%

Coenzyme Q<sub>10</sub> Levels in Maternal Plasma and Cord Blood:Correlations with Mode of Delivery

et al. 2004

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Background: Oxygen (O₂) plays a critical role in the O₂-reduction reactions indispensable for life, but can produce free radicals that are involved in many diseases. Coenzyme Q₁₀ (CoQ₁₀), acting as a redox carrier in the respiratory chain, occupies a central position in the energy metabolism and oxidative defence. Neonates seem to be very subjected to oxidative stress because of their deficient antioxidant systems. Design/Methods: The aim of the study was to verify whether the mode of delivery may affect CoQ₁₀ levels in the mother and neonate, and thus influence the risk of oxidative damage in the newborn. We measured CoQ₁₀levels in maternal plasma and cord blood at birth after three different modes of delivery (45 term healthy pregnancies): (1) vaginal in room air (VD) (n = 15); (2) elective caesarean section with general anaesthesia (50% O₂and 50% N₂O) (CSg) (n = 15), and (3) elective caesarean section with spinal anaesthesia without O₂ (CSs) (n = 15). Our results showed higher levels of Q₁₀ in mothers and neonates with VD (1.29 ± 0.43 and 0.15 ± 0.06 µg/ml, respectively) or CSs (1.15 ± 0.28 and 0.24 ± 0.06 µg/ml, respectively) when compared to CSg (0.74 ± 0.28 and 0.07 ± 0.03 µg/ml, respectively) (p < 0.01). Conclusions: These data demonstrate that the mode of delivery may affect CoQ₁₀ levels in mothers and neonates, and thus influence the risk of oxidative damage in the newborn.

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Lipophilic antioxidants in patients with phenylketonuria

Cited by 59 publications

References 20 publications

Pathologic and Immunohistochemical Findings in Hypothalamic and Mesencephalic Regions in the Pahenu2 Mouse Model for Phenylketonuria

Pathologic and Immunohistochemical Findings in Hypothalamic and Mesencephalic Regions in the Pahenu2 Mouse Model for Phenylketonuria

Oxidative Stress in Phenylketonuria: What is the Evidence?

Coenzyme Q<sub>10</sub> Levels in Maternal Plasma and Cord Blood:Correlations with Mode of Delivery

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