A murine model of neonatal diabetes mellitus in Glis3-deficient mice.

Abstract Glis3 is a member of the Gli-similar subfamily. GLIS3 mutations in humans lead to neonatal diabetes, hypothyroidism, and cystic kidney disease. We generated Glis3 -deficient mice by gene-targeting. The Glis3 −/− mice had significant increases in the basal blood sugar level during the first few days after birth. The high levels of blood sugar are attributed to a decrease in the Insulin mRNA level in the pancreas that is caused by impaired islet development and the subsequent impairment of Insulin-producing cell formation. The pancreatic phenotypes indicate that the Glis3 -deficient mice are a model for GLIS3 mutation and diabetes mellitus in humans.