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

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

Molecular Genetics and Metabolism Reports（2022）

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摘要

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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关键词

Glycogen storage disease type 1a,Atherosclerosis,Hyperlipidemia

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