Shox2-deficient mice exhibit a rare type of incomplete clefting of the secondary palate
2005
The short stature homeobox gene SHOX is associated with idiopathic
short stature in humans, as seen in Turner syndrome and Leri-Weill
dyschondrosteosis, while little is known about its close relative
SHOX2 . We report the restricted expression of Shox2 in the
anterior domain of the secondary palate in mice and humans.
Shox2 -/- mice develop an incomplete cleft that is confined
to the anterior region of the palate, an extremely rare type of clefting in
humans. The Shox2 -/- palatal shelves initiate, grow and
elevate normally, but the anterior region fails to contact and fuse at the
midline, owing to altered cell proliferation and apoptosis, leading to
incomplete clefting within the presumptive hard palate. Accompanied with these
cellular alterations is an ectopic expression of Fgf10 and
Fgfr2c in the anterior palatal mesenchyme of the mutants. Tissue
recombination and bead implantation experiments revealed that signals from the
anterior palatal epithelium are responsible for the restricted mesenchymal
Shox2 expression. BMP activity is necessary but not sufficient for
the induction of palatal Shox2 expression. Our results demonstrate an
intrinsic requirement for Shox2 in palatogenesis, and support the
idea that palatogenesis is differentially regulated along the anteroposterior
axis. Furthermore, our results demonstrate that fusion of the posterior palate
can occur independently of fusion in the anterior palate.
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