Selenium, glutathione peroxidase (GSH-Px) and lipid peroxidation products before and after selenium supplementation

Wilke, Berenice Cunha; Vidailhet, M.; Favier, A.; Guillemin, C; Ducros, Véronique; Arnaud, J; Richard, M J

doi:10.1016/0009-8981(92)90157-l

Cited by 66 publications

(41 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Glutathione plays a pivotal role in free radical detoxification, and deficiencies in this molecule have been associated with increased oxidative damage related with the pathophysiology of several diseases Plasma thiols in phenylketonuria C Colomé et al (White et al, 1994). The normal values for glutathione observed in the present study, together with the normal glutathione peroxidase activities observed in previous studies in PKU patients supplemented with selenium (Wilke et al, 1992), would suggest that the glutathione system will function in a correct way in this disease if good metabolic control is achieved.…”

Section: Discussionsupporting

confidence: 75%

Plasma thiols and their determinants in phenylketonuria

et al. 2003

View full text Add to dashboard Cite

Objective: Treatment of phenylketonuria (PKU) patients consists of a phenylalanine-restricted diet supplemented with a tyrosine-, vitamin-and oligoelement-enriched amino-acid mixture. Vitamins and oligoelements may be deficient when compliance with the supplemented special formula is poor. Plasma thiol concentrations (especially homocysteine) depend mainly on B-vitamin intake. Our aim was to evaluate the plasma thiol concentrations (homocysteine, cysteine and glutathione) and their determinants (methionine, cobalamin and folate) in PKU patients under dietary treatment compared with agematched controls. Design and setting: Cross-sectional study performed in a tertiary care Hospital. Subjects: PKU (42) patients under dietary treatment compared with 42 age-matched controls. Interventions: Plasma total homocysteine, cysteine and glutathione were analyzed by HPLC with fluorescence detection. Plasma phenylalanine and methionine were analyzed by ion exchange chromatography. Serum folate and cobalamin were analyzed by radioimmunoassay procedures. Results: Total homocysteine concentrations were significantly lower in the PKU patients compared with the control group (Students t-test; Po0.0001). Serum folate and cobalamin were significantly higher in the PKU group (t-Student; Po0.0001) compared with controls. A significantly negative correlation was observed between total homocysteine and folate (r ¼ À0.378; P ¼ 0.016), and between cobalamin and phenylalanine concentrations (r ¼ À0.367; P ¼ 0.022) in the PKU group. Conclusions: Plasma total homocysteine values are lower in the PKU group than in the controls, probably because of high folate values. High phenylalanine values, an indicator of poor dietary compliance, are negatively associated with cobalamin, which might be deficient in some cases.

show abstract

Section: Discussionsupporting

confidence: 75%

Plasma thiols and their determinants in phenylketonuria

et al. 2003

View full text Add to dashboard Cite

show abstract

“…A complementação de selênio, na dieta, normalizou as concentrações de todos os parâmetros bioquímicos relacionados ao mineral 15,16 , como encontrado no presente trabalho.…”

Section: Discussionunclassified

“…Estudos afirmam que 90 dias são suficientes para que as concentrações séricas do micronutriente, bem como as concentrações de GPX no eritrócito, aumentassem e até mesmo alcançassem os níveis da normalidade 16 . Embora se tenha verificado aumento significativo das concentrações de selênio e GPX nos eritrócitos, bem como diminuição significativa de T4L, as concentrações séricas de selênio não Tabela 2 -Médias de selênio em microgramas por dia (mcg/dia) oferecidas pelos alimentos e pela mistura de aminoácidos, de acordo com as faixas etárias estudadas Este é o único estudo brasileiro que analisa o hormônio tireoidiano (T4L) sob o ponto de vista da deficiência de selênio, além de ser o único trabalho brasileiro relatando a complementação de selênio em crianças em tratamento para PKU.…”

Section: Discussionunclassified

See 1 more Smart Citation

Selenium intake and nutritional status of children with phenylketonuria in Minas Gerais, Brazil

Alves¹,

Starling²,

Kanufre³

et al. 2012

J Pediatr (Rio J)

View full text Add to dashboard Cite

Objective: To evaluate selenium dietary intake and nutritional status of patients with phenylketonuria. Methods:The study prospectively evaluated 54 children with phenylketonuria, from 4 to10 years old. The study was performed before and after the use of a selenium-supplemented amino acid mixture. The second phase of the study was performed after, at least, 90 days of use of the supplementation. Selenium nutritional status was assessed through the analysis of biochemical parameters: serum free thyroxin and selenium and glutathione peroxidase in erythrocytes. Selenium dietary intake was evaluated by the administration of the Food Frequency Questionnaire.Results: Mean age of the children was of 7.0±1.8 years, and 35.2% were female. Mean time of supplementation of selenium, on special formula, was 122.2±25.1 days. The selenium-supplemented amino acid mixture represented 72.9% of the daily supply of the mineral. Upon supplementation, mean concentrations of serum selenium and glutathione peroxidase in erythrocytes increased significantly (p < 0.05). The average daily intake of selenium increased significantly (p < 0.001), reaching the levels recommended by the Dietary Reference Intakes. The concentration of free thyroxin, in serum, presented significant reduction (p < 0.001) in all patients during the second phase of the study, and returned to normal limits in those who had changed levels. Conclusion:Selenium supplementation through protein replacement is effective to improve and adapt the nutritional status of selenium in patients with phenylketonuria.J Pediatr (Rio J). 2012;88(5):396-400: Phenylketonuria, selenium, glutathione peroxidase, thyroid hormones. ResumoObjetivo: Avaliar a ingestão alimentar e o estado nutricional em selênio em pacientes com fenilcetonúria. Métodos:Foram avaliados prospectivamente 54 crianças com fenilcetonúria, entre 4 e 10 anos de idade. O estudo foi realizado antes e após o uso de mistura de aminoácidos complementada com selênio. A segunda fase do estudo foi realizada com, no mínimo, 90 dias de utilização da mistura complementada. O estado nutricional em selênio foi avaliado por meio da análise de parâmetros bioquímicos: dosagens séricas de selênio e tiroxina livre e dosagem de glutationa peroxidase no eritrócito. A ingestão alimentar de selênio foi avaliada por aplicação de Questionário de Frequência Alimentar Quantitativo.Resultados: A idade média das crianças foi de 7,0±1,8 anos, e 35,2% eram do sexo feminino. O tempo médio de complementação de selênio, em fórmula especial, foi de 122,2±25,1 dias. A mistura de aminoácidos complementada com o mineral representou 72,9% da oferta diária de selênio. Após a complementação, as concentrações médias de selênio sérico e de glutationa peroxidase no eritrócito apresentaram aumento significativo (p < 0,05). A ingestão média diária de selênio aumentou significativamente (p < 0,001), alcançando o recomendado pela Ingestão Dietética de Referência. A concentração de tiroxina livre, no soro, apresentou redução significativa (p < 0,001) em tod...

show abstract

“…Nevertheless, some putative side effects have been associated to this therapy, including zinc, selenium and iron deficiencies [14]. Copper, zinc [15], magnesium [16], and selenium [17] plasma levels in PKU treated children are significantly decreased, as compared to non-PKU children. The main findings presented by phenylketonuric patients are severe neurological damage, including corpus callosum, striatum, and cortical alterations and hypomyelination, that result in intellectual deficit and neurodegeneration [18][19][20][21].…”

mentioning

confidence: 99%

Phenylketonuria Pathophysiology: on the Role of Metabolic Alterations

Schuck¹,

Malgarin²,

Cararo³

et al. 2015

Aging and disease

View full text Add to dashboard Cite

Phenylketonuria (PKU) is an inborn error of phenylalanine (Phe) metabolism caused by the deficiency of phenylalanine hydroxylase. This deficiency leads to the accumulation of Phe and its metabolites in tissues and body fluids of PKU patients. The main signs and symptoms are found in the brain but the pathophysiology of this disease is not well understood. In this context, metabolic alterations such as oxidative stress, mitochondrial dysfunction, and impaired protein and neurotransmitters synthesis have been described both in animal models and patients. This review aims to discuss the main metabolic disturbances reported in PKU and relate them with the pathophysiology of this disease. The elucidation of the pathophysiology of brain damage found in PKU patients will help to develop better therapeutic strategies to improve quality of life of patients affected by this condition.

show abstract

Selenium, glutathione peroxidase (GSH-Px) and lipid peroxidation products before and after selenium supplementation

Cited by 66 publications

References 17 publications

Plasma thiols and their determinants in phenylketonuria

Plasma thiols and their determinants in phenylketonuria

Selenium intake and nutritional status of children with phenylketonuria in Minas Gerais, Brazil

Phenylketonuria Pathophysiology: on the Role of Metabolic Alterations

Contact Info

Product

Resources

About