The Rieger syndrome, an autosomal dominant disorder involving ocular, denta
l, and umbilical defects is caused by mutations in PITX2, a Bicoid-type hom
eobox protein. Mouse Pitx2 mRNA is expressed in eye, tooth and umbilicus co
nsistent with the human Riegers phenotype, Moreover, Pitx2 is involved in t
he Nodal/Sonic hedgehog pathway that determines left/right polarity. In thi
s report we demonstrate a 32-kDa polypeptide on Western blots of nuclear ex
tracts from a rat pituitary cell line, using a Pitx2 specific antibody (des
ignated P2R10), We describe also for the first time expression of the Pitx2
protein in mouse, Pitx2 protein immunostaining was detectable during the d
evelopment of the eye, tooth, umbilicus, and also in the pituitary, heart,
gut, and limb. We demonstrate for the first time directly that Pitx2 is asy
mmetrically expressed in early heart, gut, and lung development. (C) 2000 W
iley-Liss, Inc.