Pitx2, a member of the bicoid-related family of homeobox-containing ge
nes, is asymmetrically expressed in the left lateral plate mesoderm an
d derived tissues during chick and mouse development. Modifications of
Pitx2 pattern of expression in the iv mouse mutation correlate with t
he situs alterations characteristic of the mutation. Misexpression exp
eriments demonstrate that Shh and nodal positively regulate Pitx2 expr
ession. Our results are compatible with a Pitx2 function in the late p
hase of the gene cascade controlling laterality.