Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a

被引：29

作者：

Bandsma, RHJ

Rake, JP

Visser, G

Neese, RA

Hellerstein, MK

van Duyvenvoorde, W

Princen, HMG

Stellaard, F

Smit, GPA

Kuipers, F

机构：

[1] Univ Groningen Hosp, Ctr Liver Digest & Metab Dis, CMCIV, Dept Pediat, NL-9700 RB Groningen, Netherlands

[2] Univ Groningen, Inst Drug Explorat, Ctr Liver Digest & Metab Dis, NL-9700 AB Groningen, Netherlands

[3] TNO, Gaubius Lab, Leiden, Netherlands

[4] Univ Calif Berkeley, Dept Nutr Sci, Berkeley, CA 94720 USA

来源：

JOURNAL OF PEDIATRICS | 2002年 / 140卷 / 02期

关键词：

D O I：

10.1067/mpd.2002.121382

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

We describe 2 patients with glycogen storage disease type la and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the similar to40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid contents. These endings are potentially relevant for antihyperlipidemic treatment in patients with glycogen storage disease type la.

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收藏

页码：256 / 260

页数：5

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