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Hedgehog Signaling Pathway02:33

Hedgehog Signaling Pathway

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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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Notch Signaling Pathway03:14

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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.
The Notch gene came into the limelight in 1914 after the discovery that its mutation in Drosophila melanogaster leads to a serrated (or "notched") wing margin phenotype. It was not...
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Determination01:51

Determination

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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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Transducer Mechanism: G Protein–Coupled Receptors01:30

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G Protein–Coupled Receptors (GPCRs) are membrane-bound receptors that transiently associate with heterotrimeric G proteins and induce an appropriate response to various stimuli. GPCRs regulate critical physiological pathways and are excellent drug targets for treating diseases such as diabetes, cancer, obesity, depression, or Alzheimer's. Nearly 35% of approved drugs implement their therapeutic effects by selectively interacting with specific GPCRs.
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Correction: Birjandi, A.A.; Sharpe, P. The Secretome of the Inductive Tooth Germ Exhibits Signals Required for Tooth Development. <i>Bioengineering</i> 2025, <i>12</i>, 96.

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Hedgehog receptor function during craniofacial development.

Guilherme M Xavier1, Maisa Seppala1, William Barrell2

  • 1Department of Craniofacial Development and Stem Cell Biology, King's College London Dental Institute, Floor 27, Guy's Hospital, London SE1 9RT, UK; Department of Orthodontics, King's College London Dental Institute, Floor 27, Guy's Hospital, London SE1 9RT, UK.

Developmental Biology
|February 16, 2016
PubMed
Summary
This summary is machine-generated.

Sonic hedgehog (Shh) signaling is crucial for vertebrate craniofacial development, guiding forebrain and facial structure formation. Disruptions in Shh pathway components can lead to developmental defects like Holoprosencephaly.

Keywords:
BocCdonCraniofacial developmentDiseaseDispatchedEvC1/2Gas1Gpr161HoloprosencephalyLrp2/megalinPatched1Patched2Scube2SmoothenedSonic hedgehog

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Area of Science:

  • Developmental Biology
  • Molecular Biology
  • Genetics

Background:

  • The Hedgehog signalling pathway, particularly Sonic hedgehog (Shh), is vital for vertebrate craniofacial development.
  • Shh is expressed in key embryonic domains, including the ventral forebrain, facial ectoderm, and pharyngeal endoderm, influencing ectodermal and mesenchymal tissues.

Purpose of the Study:

  • To review the role of Shh signalling in early craniofacial development.
  • To focus on Hedgehog receptor function and its impact on inherited craniofacial anomalies.

Main Methods:

  • Review of existing literature on Shh signalling in craniofacial development.
  • Analysis of consequences of pathway disruption in mouse models and human populations.

Main Results:

  • Shh signalling from the prechordal plate and diencephalon is essential for forebrain division, with mutations linked to Holoprosencephaly.
  • Facial ectoderm and pharyngeal endoderm signalling influence facial development and cranial neural crest cell migration.

Conclusions:

  • Shh signalling complexity is increasing with the discovery of novel regulatory proteins.
  • Understanding Hedgehog receptor function is key to comprehending inherited craniofacial malformations.