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• W2051280817 abstract "Background and Aims Glycogen storage disease type 1a (GSD1a) is an inherited disease caused by a deficiency in the catalytic subunit of the glucose-6 phosphatase enzyme (G6Pase). GSD1a is characterized by hypoglycaemia, hyperlipidemia, and lactic acidosis with associated hepatic (including hepatocellular adenomas), renal, and intestinal disorders. A total G6pc (catalytic subunit of G6Pase) knock-out mouse model has been generated that mimics the human pathology. However, these mice rarely live longer than 3 months and long-term liver pathogenesis cannot be evaluated. Herein, we report the long-term characterization of a liver-specific G6pc knock-out mouse model (L-G6pc−/−). Methods We generated L-G6pc−/− mice using an inducible CRE-lox strategy and followed up the development of hepatic tumours using magnetic resonance imaging. Results L-G6pc−/− mice are viable and exhibit normoglycemia in the fed state. They develop hyperlipidemia, lactic acidosis, and uricemia during the first month after gene deletion. However, these plasmatic parameters improved after 6 months. L-G6pc−/− mice develop hepatomegaly with glycogen accumulation and hepatic steatosis. Using an MRI approach, we could detect hepatic nodules with diameters of less than 1 mm, 9 months after induction of deficiency. Hepatic nodules (1 mm) were detected in 30–40% of L-G6pc−/− mice at 12 months. After 18 months, all L-G6pc−/− mice developed multiple hepatocellular adenomas of 1–10 mm diameter. Conclusions This is the first report of a viable animal model of the hepatic pathology of GSD1a, including the late development of hepatocellular adenomas. Glycogen storage disease type 1a (GSD1a) is an inherited disease caused by a deficiency in the catalytic subunit of the glucose-6 phosphatase enzyme (G6Pase). GSD1a is characterized by hypoglycaemia, hyperlipidemia, and lactic acidosis with associated hepatic (including hepatocellular adenomas), renal, and intestinal disorders. A total G6pc (catalytic subunit of G6Pase) knock-out mouse model has been generated that mimics the human pathology. However, these mice rarely live longer than 3 months and long-term liver pathogenesis cannot be evaluated. Herein, we report the long-term characterization of a liver-specific G6pc knock-out mouse model (L-G6pc−/−). We generated L-G6pc−/− mice using an inducible CRE-lox strategy and followed up the development of hepatic tumours using magnetic resonance imaging. L-G6pc−/− mice are viable and exhibit normoglycemia in the fed state. They develop hyperlipidemia, lactic acidosis, and uricemia during the first month after gene deletion. However, these plasmatic parameters improved after 6 months. L-G6pc−/− mice develop hepatomegaly with glycogen accumulation and hepatic steatosis. Using an MRI approach, we could detect hepatic nodules with diameters of less than 1 mm, 9 months after induction of deficiency. Hepatic nodules (1 mm) were detected in 30–40% of L-G6pc−/− mice at 12 months. After 18 months, all L-G6pc−/− mice developed multiple hepatocellular adenomas of 1–10 mm diameter. This is the first report of a viable animal model of the hepatic pathology of GSD1a, including the late development of hepatocellular adenomas." @default.
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• W2051280817 date "2011-03-01" @default.
• W2051280817 modified "2023-10-18" @default.
• W2051280817 title "Targeted deletion of liver glucose-6 phosphatase mimics glycogen storage disease type 1a including development of multiple adenomas" @default.
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• W2051280817 doi "https://doi.org/10.1016/j.jhep.2010.08.014" @default.
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