The role of Shoc2 in differentiation processes could explain both the effects of a dominant mutant form of this protein in developmental disorders such as the Noonan-like syndrome [15], and the defects of the atrioventricular canal that cause early embryonic lethality when the Shoc2/Sur8 gene is inactivated in mouse endothelial cells [46]. The gene discussed is SHOC2; the disease is Noonan syndrome.