Sonic hedgehog signaling in craniofacial development

Mutations in SHH and several other genes encoding components of the Hedgehog signaling pathway have been associated with holoprosencephaly syndromes, with craniofacial anomalies ranging in severity from cyclopia to facial cleft to midfacial and mandibular hypoplasia. Studies in animal models have revealed that SHH signaling plays crucial roles at multiple stages of craniofacial morphogenesis, from cranial neural crest cell survival to growth and patterning of the facial primordia to organogenesis of the palate, mandible, tongue, tooth, and taste bud formation and homeostasis. This article provides a summary of the major findings in studies of the roles of SHH signaling in craniofacial development, with emphasis on recent advances in the understanding of the molecular and cellular mechanisms regulating the SHH signaling pathway activity and those involving SHH signaling in the formation and patterning of craniofacial structures.

Automated summary

Mutations in SHH and other genes in the Hedgehog signaling pathway are associated with holoprosencephaly syndromes and craniofacial anomalies. SHH signaling plays crucial roles in craniofacial morphogenesis, including cell survival, growth and patterning, and organogenesis of various structures. This article summarizes recent advances in understanding the molecular and cellular mechanisms of SHH signaling and its involvement in the formation and patterning of craniofacial structures.