Molecular Genetics and Metabolism Reports (Jun 2022)

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

Journal volume & issue
Vol. 31
p. 100872

Abstract

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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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