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
Affiliations
- Anouk M. La Rose
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Anouk G. Groenen
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Benedek Halmos
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Venetia Bazioti
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Martijn G.S. Rutten
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Kishore A. Krishnamurthy
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Mirjam H. Koster
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Niels J. Kloosterhuis
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Marieke Smit
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Rick Havinga
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Gilles Mithieux
- Université Claude Bernard Lyon 1, Université de Lyon, INSERM UMR-S1213, Lyon, France
- Fabienne Rajas
- Université Claude Bernard Lyon 1, Université de Lyon, INSERM UMR-S1213, Lyon, France
- Folkert Kuipers
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands; Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Maaike H. Oosterveer
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
- Marit Westerterp
- Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands; Corresponding author at: Department of Pediatrics, University Medical Center Groningen, ERIBA Building 3226 room 04.14, Antonius Deusinglaan 1, 9713 AV Groningen, the Netherlands.
- Journal volume & issue
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Vol. 31
p. 100872
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.