Increased atherosclerosis in a mouse model of glycogen storage disease type 1a

1a 型糖原累积病小鼠模型中动脉粥样硬化增加

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作者:Anouk M La Rose, Anouk G Groenen, Benedek Halmos, Venetia Bazioti, Martijn G S Rutten, Kishore A Krishnamurthy, Mirjam H Koster, Niels J Kloosterhuis, Marieke Smit, Rick Havinga, Gilles Mithieux, Fabienne Rajas, Folkert Kuipers, Maaike H Oosterveer, Marit Westerterp

Abstract

Glycogen storage disease type 1a (GSD Ia) is an inborn error of carbohydrate metabolism. Despite severe hyperlipidemia, GSD Ia patients show limited atherogenesis compared to age-and-gender matched controls. Employing a GSD Ia mouse model that resembles the severe hyperlipidemia in patients, we here found increased atherogenesis in GSD Ia. These data provide a rationale for investigating atherogenesis in GSD Ia in a larger patient cohort.

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