Increased atherosclerosis in a mouse model of glycogen storage disease type 1a
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.
Keywords
Atherosclerosis
G6P
Glucose-6-phosphate
G6PC1
Glucose-6-phosphatase enzyme
GSD Ia
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.
Domains
Neurons and Cognition [q-bio.NC]
Origin : Publication funded by an institution