Ege University Faculty of Medicine, Department of Pediatric Endocrinology and Metabolism, 35100, Bornova, Izmir, Turkey.
J Trace Elem Med Biol. 2010 Jan;24(1):42-5. doi: 10.1016/j.jtemb.2009.10.004. Epub 2009 Nov 24.
Dyslipidemia in patients with glycogen storage disease types Ia (GSD Ia) and III (GSD III) does not lead to premature atherosclerosis. The aim of this study was to investigate the association among serum copper (Cu), zinc (Zn), iron (Fe), and selenium (Se) concentrations, and their carrier proteins: ceruloplasmin, albumin, and related antioxidant enzyme activities [superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), paraoxonase (PON), and arylesterase (ARYL)] in 20 GSD Ia and 14 III patients compared to age and sex matched 20 healthy subjects. Erythrocyte oxidative stress was measured by erythrocyte thiobarbituric acid reactive substances (eTBARSs). Hypertriglyceridemia [333 (36-890)mg/dL] in GSD Ia and hypercholesterolemia with elevated LDL-cholesterol [188 (91-313)mg/dL] and decreased HDL-cholesterol [32(23-58)mg/dL] levels in GSD III were found. Serum Cu, Fe, and Zn showed no significant differences between groups. However, Se 60 (54-94), 81 (57-127) microg/L, ceruloplasmin 21 (10-90), 27 (23-65) microg/L, and albumin 2.4 (1.7-5.1), 2.8 (1.8-4.06)g/dL levels were decreased in GSD Ia and III groups, respectively, in comparison with the controls [Se 110 (60-136) microg/L, ceruloplasmin 72 (32-94) microg/L, and albumin 4.4 (4-4.8)g/dL)]. In spite of high oxidative stress in erythrocyte detected by elevated eTBARS/Hb levels in GSD group [674.8 (454.6-948.2) for GSD Ia, 636.3 (460.9-842.1) for GSD III, and 525.6 (449.2-612.6)], the activities of CAT, SOD, ARYL, and PON in GSD patients were not different from the controls. GPx activity was decreased in GSD Ia [3.7 (1.8-7.1)U/mL] and GSD III [4.2 (2.2-8.6)U/mL] compared with healthy controls [7.1 (2.9-16.2)U/mL]. In conclusion, this study supplied the data for trace elements, their carrier, and antioxidative enzymes in the patients with GSD Ia and III. The trace elements and anti-oxidative enzyme levels in GSD patients failed to explain the atherosclerotic escape phenomenon reported in these patients.
糖原贮积病 Ia 型（GSD Ia）和 III 型（GSD III）患者的血脂异常不会导致动脉粥样硬化提前发生。本研究旨在探讨 20 例 GSD Ia 患者和 14 例 GSD III 患者与年龄和性别相匹配的 20 例健康受试者之间血清铜（Cu）、锌（Zn）、铁（Fe）和硒（Se）浓度及其载体蛋白[铜蓝蛋白、白蛋白和相关抗氧化酶活性[超氧化物歧化酶（SOD）、过氧化氢酶（CAT）、谷胱甘肽过氧化物酶（GPx）、对氧磷酶（PON）和芳基酯酶（ARYL）]之间的关系。通过红细胞硫代巴比妥酸反应物质（eTBARSs）测量红细胞氧化应激。发现 GSD Ia 患者存在高三酰甘油血症[333（36-890）mg/dL]，GSD III 患者存在高胆固醇血症伴 LDL-胆固醇升高[188（91-313）mg/dL]和 HDL-胆固醇降低[32（23-58）mg/dL]。各组间血清 Cu、Fe 和 Zn 无显著差异。然而，GSD Ia 和 III 组的 Se 水平[60（54-94）、81（57-127）μg/L]、铜蓝蛋白水平[21（10-90）、27（23-65）μg/L]和白蛋白水平[2.4（1.7-5.1）、2.8（1.8-4.06）g/dL]均较对照组降低[Se 110（60-136）μg/L、铜蓝蛋白 72（32-94）μg/L 和白蛋白 4.4（4-4.8）g/dL]。尽管 GSD 组红细胞中的 eTBARS/Hb 水平升高表明氧化应激较高[GSD Ia 组 674.8（454.6-948.2），GSD III 组 636.3（460.9-842.1），对照组 525.6（449.2-612.6）]，但 GSD 患者的 CAT、SOD、ARYL 和 PON 活性与对照组无差异。GSD Ia [3.7（1.8-7.1）U/mL]和 GSD III [4.2（2.2-8.6）U/mL]患者的 GPx 活性低于健康对照组[7.1（2.9-16.2）U/mL]。总之，本研究为 GSD Ia 和 III 患者提供了微量元素、其载体和抗氧化酶的数据。GSD 患者的微量元素和抗氧化酶水平无法解释这些患者报告的动脉粥样硬化逃逸现象。
Zotero 插件