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Related Concept Videos

Canonical Wnt Signaling Pathway02:54

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The gene encoding the main signaling molecules of the Wnt signaling pathways (the Wnt proteins) was discovered almost four decades ago by Nüsslein-Volhard and Wieschaus. They identified and originally named the gene "wingless" (wg) after a phenotype discovered during their landmark genetic screen in Drosophila for body pattern defects. At around the same time, another researcher named Harold Varmus found that a murine tumor virus activates the mammalian wg homolog, Int-1, which...
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Wnt is a zygotic effect gene that is expressed during very early embryonic development. It regulates various processes in animals starting from early development through the adult stage, such as organogenesis in the embryo and maintenance of neuronal and blood stem cells. Wnt proteins can induce a wide variety of intracellular pathways depending upon the specific abilities of different Wnt ligands to form a complex with shared and cognate receptors in the presence of different co-receptors. The...
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Catenins are characterized by multiple binding domains and dynamic structures that allow them to function as linker proteins in cell junction complexes. All catenins, except α-catenin, contain a characteristic protein sequence called the armadillo repeat and are therefore also called armadillo proteins.
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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.
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Hedgehog Signaling Pathway02:33

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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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TGF - β Signaling Pathway01:16

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The TGF-β signaling pathway regulates cell growth, differentiation, adhesion, motility, and development. TGF-β ligands that induce TGF-β signaling are synthesized in their latent form. Several proteases or cell surface receptors such as integrins act upon the latent form, releasing the active ligand. There are three types of mammalian TGF-βs: (TGF-β1, TGF-β2, and TGF-β3) that bind as homodimers or heterodimers to TGF-β receptors. The TGF-β receptors...
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Updated: Oct 4, 2025

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis
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Wnt/β-catenin Signaling Controls Maxillofacial Hyperostosis.

J Chen1,2, P L Cuevas1, J S Dworan1,3

  • 1Division of Plastic and Reconstructive Surgery, Department of Surgery, Stanford University School of Medicine, Palo Alto, CA, USA.

Journal of Dental Research
|February 4, 2022
PubMed
Summary
This summary is machine-generated.

Constitutively active Wnt/β-catenin signaling in mice causes progressive craniomaxillofacial bone overgrowth and abnormal matrix. This highlights a feedback loop impacting bone development and potentially human craniotubular disorders.

Keywords:
Wnt signaling pathwaycongenital cortical hyperostosiscraniofacial abnormalitiescraniotubular disordersfacial bonesperiosteum

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Area of Science:

  • Bone biology
  • Craniomaxillofacial development
  • Signaling pathways

Background:

  • Wnt/β-catenin signaling is crucial for bone homeostasis.
  • Dysregulation can lead to skeletal abnormalities.
  • Craniomaxillofacial (CMF) bone development is complex.

Purpose of the Study:

  • To investigate the role of Wnt/β-catenin signaling in CMF bone morphology and microstructure.
  • To elucidate the cellular and molecular mechanisms underlying Wnt/β-catenin-induced CMF bone phenotypes.

Main Methods:

  • Utilized transgenic mice (daβcatOt) with constitutively active β-catenin in Dmp1-expressing cells.
  • Conducted molecular and cellular analyses at postnatal day 24 and beyond.
  • Examined bone morphology, microstructure, and mineralization.

Main Results:

  • daβcatOt mice showed midfacial truncations and progressive maxillary/mandibular hyperostosis.
  • Increased osteoblast number and mineral apposition rate observed.
  • Abnormal CMF bone matrix with excess osteoid and woven bone formation, alongside immature osteocytes.

Conclusions:

  • Unrestrained Wnt/β-catenin signaling drives a ligand-independent positive feedback loop.
  • This results in progressive CMF hyperostosis with architecturally abnormal, poorly mineralized bone.
  • The phenotype resembles human craniotubular disorders, suggesting a shared pathway.