Targeted deletion of liver glucose-6 phosphatase mimics glycogen storage disease type 1a including development of multiple adenomas.

Elodie Mutel (1) , Aya Abdul-Wahed (1) , Nirilanto Ramamonjisoa (2) , Anne Stefanutti (1) , Isabelle Houberdon (1) , Sophie Cavassila (2) , Frank Pilleul (1) , Olivier Beuf (2) , Amandine Gautier-Stein (1) , Armelle Penhoat (1) , Gilles Mithieux (1) , Fabienne Rajas (1)

1 Nutrition et cerveau (U1213, U855)
2 RMN et optique : De la mesure au biomarqueur

This is the first report of a viable animal model of the hepatic pathology of GSD1a, including the late development of hepatocellular adenomas.

Domaines: Neurosciences [q-bio.NC]

Elodie Mutel, Aya Abdul-Wahed, Nirilanto Ramamonjisoa, Anne Stefanutti, Isabelle Houberdon, et al.. Targeted deletion of liver glucose-6 phosphatase mimics glycogen storage disease type 1a including development of multiple adenomas.. Journal of Hepatology, 2011, 54 (3), pp.529-37. ⟨10.1016/j.jhep.2010.08.014⟩. ⟨hal-00575314⟩