Therefore, our animal model for GSD-1a is unique because: (i) it is the only model with a constitutive liver deletion of G6pc that allows the high mortality of the knockout model to be overcome (Lei et al., 1996) and permits the study of the GSD-1a liver disease without delaying the onset of the deletion; and (ii) it is the only one so far described that mimics all the steps of the disease progression, including the development of HCC, providing experimentally a link between G6Pase-α deficiency and neoplastic liver progression. Here, G6PC1 is linked to liver disorder.