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Ectopic Hedgehog Signaling Causes Cleft Palate and Defective Osteogenesis

N L Hammond1, K J Brookes1,2, M J Dixon1

  • 11 Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK.

Journal of Dental Research
|July 6, 2018
PubMed

Insights

Sonic Hedgehog (SHH) signaling disruption causes cleft palate by affecting gene networks. Ectopic SHH signaling impairs bone formation and Wnt/BMP pathways, leading to this common birth defect.

Area of Science:

  • Developmental Biology
  • Genetics
  • Craniofacial Development

Background:

  • Cleft palate is a frequent congenital malformation.
  • Mutations in Sonic Hedgehog (SHH) signaling pathways are implicated in cleft palate.
  • The gene regulatory networks downstream of Hedgehog (Hh) signaling are not fully understood.

Purpose of the Study:

  • To investigate the downstream gene regulatory networks of Hh signaling in secondary palate development.
  • To elucidate the mechanisms by which Hh signaling influences palatal bone formation and patterning.
  • To identify key transcription factors and signaling pathways regulated by Hh.

Main Methods:

  • Ectopic Hh signaling was induced in the palatal mesenchyme.
  • Analysis of oral-nasal patterning of neural crest-derived ectomesenchyme.
  • Investigation of Fox transcription factors and Wnt/BMP antagonists, including Sostdc1.
  • Assessment of osteogenesis and BMP signaling effectors.

Main Results:

  • Ectopic Hh signaling disrupted palatal shelf patterning and led to cleft palate.
  • Fox transcription factors, including Foxl1, were identified as downstream targets of Hh signaling.
  • Hh signaling positively regulated Wnt/BMP antagonists like Sostdc1.
  • Hh signaling downregulated osteogenesis regulators and BMP signaling effectors.

Conclusions:

  • Ectopic Hh signaling disrupts craniofacial development, causing cleft palate.
  • Hh signaling regulates key transcription factors and antagonists in palate development.
  • Hh-Smo signaling downregulates Wnt/BMP pathways, partly via Sostdc1, leading to defective osteogenesis and cleft palate.

Keywords:
bone morphogenetic proteinscraniofacial abnormalitiesmicrognathismneural crestsonic hedgehogwnt signaling pathway

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