In summation, Tie2Cre/Nrp1 mutants have a high or complete penetrance of cardiac outflow tract, great vessel, pharyngeal organ, and craniofacial defects that collectively typify DiGeorge syndrome in humans and that are also seen with high or complete penetrance in Tbx1 and Vegfa mutant mice. Here, NRP1 is linked to 22q11.2 deletion syndrome.