Increased atherosclerosis in a mouse model of glycogen storage disease type 1a.
Mol Genet Metab Rep
; 31: 100872, 2022 Jun.
Article
en En
| MEDLINE
| ID: mdl-35782606
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.
Atherosclerosis; G6P, Glucose-6-phosphate; G6PC1, Glucose-6-phosphatase enzyme; GSD Ia, Glycogen storage disease type 1a; Glycogen storage disease type 1a; HDL, High-density lipoprotein; Hyperlipidemia; Ldlr−/−, LDL receptor deficient; PUFA, Poly-unsaturated FA; SFA, Saturated fatty acids; TG, Triglycerides; VLDL, Very-low-density lipoprotein; WTD, Western-type diet
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MEDLINE
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En
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Mol Genet Metab Rep
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2022
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Article
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Países Bajos