Advanced glycation end products and the absence of premature atherosclerosis in glycogen storage disease Ia
Advanced glycation end products and the absence of premature atherosclerosis in glycogen storage disease Ia
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DOI：
10.1007/s10545-007-0507-0
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发表时间：
2007-11-01
影响因子：
4.2
通讯作者：
Smit, A. J.
中科院分区：
文献类型：
--
作者：
den Hollander, N. C.;Mulder, D. J.;Smit, A. J.
产品介绍：尽管有不利的心血管风险，但Ia型糖原累积病（GSD Ia）患者不会过早发生动脉粥样硬化。我们推测这种矛盾可能与终生低血糖水平和氧化应激降低导致晚期糖基化终产物（AGEs）形成减少有关。研究方法：在8例GSD Ia患者中（年龄20-34岁）和30名匹配的对照者，我们测量了颈动脉内膜中层厚度（IMT）、皮肤自发荧光（（AF; AGEs的非侵入性指数）和特定AGEs（戊糖苷，N-（羧甲基）赖氨酸（CML），N-（羧乙基）赖氨酸（CEL））和胶原连接荧光（CLF，在328/378和370/440 nm的激发/发射波长组合下测量）。结果：GSD Ia患者颈动脉IMT明显降低。皮肤AF在患者和对照组之间没有差异。皮肤样本显示患者组中的CEL水平较高（p=0.008），但戊糖苷、CML和CLF水平相似。在总组中，皮肤AF与CML（r=0.39，p=0.031）、CLF 328/378 nm（r=0.53; p=0.002）和CLF 370/440 nm（r=0.60; p=0.001）相关。在对照组中，AF也与最大颈动脉IMT相关（r=0.6; p=0.004）。结论：虽然我们的数据证实GSD Ia患者动脉粥样硬化的负担减轻，但这种现象不能用皮肤中AGE积累的差异来解释。
Introducton: Despite their unfavourable cardiovascular risk profile, patients with glycogen storage disease type Ia (GSD Ia) do not develop premature atherosclerosis. We hypothesized that this paradox might be related to a decreased formation of advanced glycation end products (AGEs) resulting from lifetime low plasma glucose levels and decreased oxidative stress. Methods: In 8 GSD Ia patients (age 20-34 years) and 30 matched controls we measured carotid intima-media thickness (IMT), skin autofluorescence (AF; a non-invasive index for AGEs), and specific AGEs (pentosidine, N-(carboxymethyl)lysine (CML), N-(carboxyethyl)lysine (CEL)) and collagen linked fluorescence (CLF, measured at excitation/emission wavelength combinations of 328/378 and 370/440 nm) in skin samples. Results: Carotid IMT was significantly lower in GSD Ia patients. Skin AF did not differ between patients and controls. The skin samples showed higher CEL levels in the patient group (p=0.008), but similar levels of pentosidine, CML, and CLF. In the total group, skin AF correlated with CML (r=0.39, p=0.031), CLF 328/378 nm (r=0.53; p=0.002) and CLF 370/440 nm (r=0.60; p=0.001). In the control group, AF also correlated with the maximum carotid IMT (r=0.6; p=0.004). Conclusion: Although our data confirm that GSD Ia patients present with a reduced burden of atherosclerosis, this phenomenon cannot be explained by differences in AGE accumulation as measured in the skin.