Currently, there have been studies of SUR1 knockout mice in a variety of pathological conditions, such as persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) (Miki et al., 1999) and a severe dysregulation of glucose homeostasis known as congenital hyperinsulinism (CHI) (Straub et al., 2001; Dunne et al., 2004; Bryan et al., 2007; Nakamura & Bryan, 2014). The gene discussed is ABCC8; the disease is congenital isolated hyperinsulinism.