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The Hedgehog gene (Hh) was first discovered due to its control of the growth of disorganized, hair-like bristles phenotype in Drosophila, much like hedgehog spines. Hh plays a crucial role in the development of organs and the maintenance of homeostasis in both invertebrates and vertebrates. However, while Drosophila has only one Hh protein, mammals have multiple functional Hedgehog proteins - Sonic (Shh), Desert (Dhh), and Indian Hedgehog (Ihh). All of these homologous proteins have adapted to...
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During embryogenesis, cells become progressively committed to different fates through a two-step process: specification followed by determination. Specification is demonstrated by removing a segment of an early embryo, “neutrally” culturing the tissue in vitro—for example, in a petri dish with simple medium—and then observing the derivatives. If the cultured region gives rise to cell types that it would normally generate in the embryo, this means that it is specified. In...
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The Notch signaling pathway is a major intracellular signaling pathway that is highly conserved over a broad spectrum of metazoan species. It stands unique from other intracellular signaling mechanisms in animals because notch protein itself acts as the receptor as well as the primary signaling molecule.
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Sonic hedgehog signaling in craniofacial development.

Jingyue Xu1, Paul P R Iyyanar1, Yu Lan2

  • 1Division of Developmental Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, USA.

Differentiation; Research in Biological Diversity
|July 23, 2023
PubMed
Summary
This summary is machine-generated.

Mutations in the Hedgehog signaling pathway, particularly SHH, cause holoprosencephaly and craniofacial defects. This pathway is vital for facial development, from cell survival to organ formation.

Keywords:
MandibleNeural crestPalatePrimary ciliumShhTonguecholesterol

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Area of Science:

  • Developmental Biology
  • Genetics
  • Craniofacial Biology

Background:

  • Mutations in the Sonic Hedgehog (SHH) gene and other Hedgehog pathway components are linked to holoprosencephaly syndromes.
  • These syndromes manifest in craniofacial anomalies of varying severity, including cyclopia, facial clefts, and hypoplasia of the midface and mandible.

Purpose of the Study:

  • To summarize key findings on the role of SHH signaling in craniofacial development.
  • To highlight recent advances in understanding the molecular and cellular mechanisms regulating SHH pathway activity and its impact on craniofacial structures.

Main Methods:

  • Review of existing literature on SHH signaling and craniofacial development.
  • Analysis of studies in animal models demonstrating SHH's role in morphogenesis.

Main Results:

  • SHH signaling is essential for multiple stages of craniofacial morphogenesis.
  • It influences cranial neural crest cell survival, facial primordia growth and patterning, and organogenesis of the palate, mandible, tongue, teeth, and taste buds.

Conclusions:

  • SHH signaling is a critical regulator of normal craniofacial development.
  • Understanding its molecular mechanisms provides insights into congenital craniofacial anomalies.