کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2787027 1568437 2009 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Effect of short- and long-term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شناسی تکاملی
پیش نمایش صفحه اول مقاله
Effect of short- and long-term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients
چکیده انگلیسی

Phenylketonuria is the most frequent disturbance of amino acid metabolism. Treatment for phenylketonuric patients consists of phenylalanine intake restriction. However, there are patients who do not adhere to treatment and/or are not submitted to neonatal screening. These individuals are more prone to develop brain damage due to long-lasting toxic effects of high levels of phenylalanine and/or its metabolites. Oxidative stress occurs in late-diagnosed phenylketonuric patients, probably contributing to the neurological damage in this disorder. In this work, we aimed to compare the influence of time exposition to high phenylalanine levels on oxidative stress parameters in phenylketonuric patients who did not adhere to protein restricted diet. We evaluated a large spectrum of oxidative stress parameters in plasma and erythrocytes from phenylketonuric patients with early and late diagnosis and of age-matched healthy controls. Erythrocyte glutathione peroxidase activity and glutathione levels, as well as plasma total antioxidant reactivity were significantly reduced in both groups of patients when compared to the control group. Furthermore, protein oxidative damage, measured by carbonyl formation and sulfhydryl oxidation, and lipid peroxidation, determined by malondialdehyde levels, were significantly increased only in patients exposed for a long time to high phenylalanine concentrations, compared to early diagnosed patients and controls. In conclusion, exposition to high phenylalanine concentrations for a short or long time results in a reduction of non-enzymatic and enzymatic antioxidant defenses, whereas protein and lipid oxidative damage only occurs in patients with late diagnosis.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: International Journal of Developmental Neuroscience - Volume 27, Issue 3, May 2009, Pages 243–247
نویسندگان
, , , , , , , , , ,