G6PC1 and familial hypercholesterolemia: We show that the huR83C mice lack hepatic and renal G6Pase-α activity and manifest a phenotype of impaired glucose homeostasis mimicking GSD-Ia patients1–3, characterized by fasting hypoglycemia, growth retardation, hepatomegaly, nephromegaly, hypertriglyceridemia, hypercholesterolemia, and hyperuricemia.