Mice with an anterior cleft of the palate survive neonatal lethality.
Many genes are known to function in a region-specific manner in the developing secondary palate. We have previously shown that Shox2-deficient embryos die at mid-gestation stage and develop an anterior clefting phenotype. Here, we show that mice carrying a conditional inactivation of Shox2 in the palatal mesenchyme survive the embryonic and neonatal lethality, but develop a wasting syndrome. Phenotypic analyses indicate a delayed closure of the secondary palate at the anterior end, leading to a failed fusion of the primary and secondary palates. Consistent with a role proposed for Shox2 in skeletogenesis, Shox2 inactivation causes a significantly reduced bone formation in the hard palate, probably due to a down-regulation of Runx2 and Osterix. We conclude that the secondary palatal shelves are capable of fusion with each other, but fail to fuse with the primary palate in a developmentally delayed manner. Mice carrying an anterior cleft can survive neonatal lethality.
Gu
Shuping
S
Section of Oral Biology, The Ohio State University College of Dentistry, Columbus, Ohio 43210, USA.
Wei
Na
N
Yu
Xueyan
X
Jiang
Yiping
Y
Fei
Jian
J
Chen
YiPing
Y
D000818:Animals; D002972:Cleft Palate / Q000235:genetics / Q000401:mortality; D051526:Fibroblast Growth Factor 10 / Q000235:genetics / Q000378:metabolism; D006609:High Mobility Group Proteins / Q000235:genetics / Q000378:metabolism; D018398:Homeodomain Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D017403:In Situ Hybridization; D008648:Mesoderm / Q000166:cytology / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D010012:Osteogenesis / Q000502:physiology; D010159:Palate / Q000002:abnormalities / Q000033:anatomy & histology* / Q000196:embryology*; D010641:Phenotype; D055758:SOX9 Transcription Factor; D015996:Survival Rate; D046509:Tissue Culture Techniques; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism
C496464:Fgf10 protein, mouse; D051526:Fibroblast Growth Factor 10; D006609:High Mobility Group Proteins; D018398:Homeodomain Proteins; D055758:SOX9 Transcription Factor; C527932:SOX9 protein, human; C484663:Shox2 protein, mouse; C527933:Sox9 protein, mouse; D014157:Transcription Factors