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Related Experiment Videos

Sonic hedgehog is essential for first pharyngeal arch development.

Chihiro Yamagishi1, Hiroyuki Yamagishi, Jun Maeda

  • 1Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.

Pediatric Research
|February 24, 2006
PubMed
Summary

Sonic hedgehog (Shh) signaling is crucial for craniofacial development. Shh deficiency in mouse embryos impairs first pharyngeal arch development by increasing apoptosis and downregulating Fgf8, essential for tissue growth.

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Area of Science:

  • Developmental Biology
  • Molecular Biology
  • Genetics

Background:

  • Sonic hedgehog (Shh) is a secreted protein vital for organogenesis.
  • Shh disruption in mice causes craniofacial defects, and SHH mutations in humans lead to holoprosencephaly (HPE) and pharyngeal arch abnormalities.

Purpose of the Study:

  • To investigate the role of Shh signaling in early pharyngeal arch development.
  • To analyze the impact of Shh deficiency on the first pharyngeal arch (PA1) in mouse embryos.

Main Methods:

  • Analysis of Shh mutant mouse embryos using molecular markers.
  • TUNEL assay to detect apoptosis.
  • Examination of gene expression for fibroblast growth factor (Fgf)-8 and its downstream targets.

Main Results:

Related Experiment Videos

  • Shh-null PA1 embryos exhibited hypoplasia and midline fusion of the first pharyngeal arch.
  • Maxillary and proximal mandibular arch growth were severely defective, while the distal mandibular arch was less affected.
  • Increased apoptosis and down-regulation of Fgf8 and its downstream targets (Barx1, goosecoid, Dlx2) were observed in Shh-null PA1.

Conclusions:

  • Shh signaling is essential for PA1 development, regulating cell survival and tissue outgrowth.
  • Shh coordinates epithelial-mesenchymal signaling and transcriptional events, partly through Fgf8.
  • Defects in Shh signaling disrupt Fgf8-dependent pathways critical for PA1 formation.