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β重型地中海贫血患者的氨基酸和脂肪酸。

Amino acids and fatty acids in patients with beta thalassemia major.

机构信息

Suleyman Demirel University.

.

出版信息

Acta Biomed. 2020 Mar 19;91(1):79-84. doi: 10.23750/abm.v91i1.7266.

DOI:10.23750/abm.v91i1.7266
PMID:32191658
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7569589/
Abstract

BACKGROUND

Oxidative damage and increasing of lipid peroxidation are caused by chronic iron overload in patients with beta thalassemia major. Fatty acids are important structural elements for palmitoylation of membrane proteins which constitute a great part of natural membranes. Oxidative damages caused by reactive oxygen derives in thalassemic erythrocytes can be determined with lipid peroxidation, protein oxidation, and antioxidant system elements. The aim of study was to evaluate the relationship between amino acid and fatty acid levels with iron overload and antioxidant enzymes in beta thalassemia major.

METHODS

A total 40 patients with beta thalassemia major with regular blood transfusion and chelating agents were included in the study. The levels of serum amino acid, fatty acid, ferritin, antioxidant enzymes and malondialdehyde were measured.

RESULTS

Only C16- palmitoyl level was found significantly low in patients, other fatty acids and amino acids were in normal range. There were lower malondialdehyde and ferritin levels in patients with low C-16 palmitoyl level (p<0.05).  Conclusions: The high levels of ferritin and malondialdehyde in the patients with low C16-palmitoyl levels might be caused by this fatty acid's preventative effect on oxidative stress.

摘要

背景

β地中海贫血症患者由于慢性铁过载会导致氧化损伤和脂质过氧化增加。脂肪酸是膜蛋白棕榈酰化的重要结构元素,而膜蛋白构成了天然膜的很大一部分。在β地中海贫血症患者的红细胞中,由活性氧衍生的氧化损伤可以通过脂质过氧化、蛋白质氧化和抗氧化系统元素来确定。本研究旨在评估β地中海贫血症患者中氨基酸和脂肪酸水平与铁过载和抗氧化酶之间的关系。

方法

本研究共纳入 40 名定期输血和使用螯合剂的β地中海贫血症患者。测量了血清氨基酸、脂肪酸、铁蛋白、抗氧化酶和丙二醛的水平。

结果

仅发现患者的 C16-棕榈酰水平显著降低,其他脂肪酸和氨基酸均在正常范围内。低 C-16 棕榈酰水平患者的丙二醛和铁蛋白水平较低(p<0.05)。

结论

低 C16-棕榈酰水平患者的铁蛋白和丙二醛水平较高,可能是由于这种脂肪酸对氧化应激具有预防作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ead/7569589/86f5d882113e/ACTA-91-79-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ead/7569589/86f5d882113e/ACTA-91-79-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ead/7569589/86f5d882113e/ACTA-91-79-g001.jpg

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