Related Experiment Video
Updated: Dec 27, 2025
07:26
Quantitative PCR-based Assay to Measure Sonic Hedgehog Signaling in Cellular Model of Ciliogenesis
Published on: January 31, 2025
940
SHH Signaling Pathway Drives Pediatric Bone Sarcoma Progression.
Frédéric Lézot1, Isabelle Corre1, Sarah Morice1
1INSERM UMR1238, PHY-OS, "Bone sarcomas and remodeling of calcified tissues", Nantes University, 44000 Nantes, France.
Cells
|March 1, 2020
Summary
Pediatric bone cancers like osteosarcoma and Ewing sarcoma hijack bone remodeling. This review explores the Sonic Hedgehog (SHH) pathway
Area of Science:
- Oncology
- Molecular Biology
- Skeletal Biology
Background:
- Malignant primary bone tumors, including osteosarcoma and Ewing sarcoma, are prevalent in children and adolescents.
- Despite advancements, treatment outcomes for metastatic bone sarcomas remain poor, with high mortality rates.
- These bone tumors uniquely deregulate bone homeostasis and remodeling to promote their growth.
Purpose of the Study:
- To review current knowledge on the Sonic Hedgehog (SHH) signaling pathway's role in skeletal development.
- To elucidate the involvement of the SHH pathway in pediatric bone sarcoma progression.
- To discuss potential therapeutic strategies targeting the SHH pathway for bone sarcomas.
Main Methods:
- Literature review of scientific publications.
- Analysis of studies on skeletal development and bone tumor biology.
- Examination of research on SHH pathway activation (canonical and non-canonical).
Main Results:
- The SHH signaling pathway is implicated in normal skeletal development.
- Evidence suggests SHH pathway dysregulation contributes to pediatric bone sarcoma progression.
- Both canonical and non-canonical SHH activation mechanisms are relevant in cancer.
Conclusions:
- The SHH pathway is a critical player in both skeletal development and bone sarcoma pathogenesis.
- Targeting the SHH pathway presents a promising therapeutic avenue for pediatric bone sarcomas.
- Further research is needed to fully understand and exploit SHH signaling for treatment.
Related Concept Videos
02:33Hedgehog Signaling Pathway
9.7K
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...
9.7K
03:03mTOR Signaling and Cancer Progression
4.5K
The mammalian target of rapamycin or mTOR protein was discovered in 1994 due to its direct interaction with rapamycin. The protein gets its name from a yeast homolog called TOR. The mTOR protein complex in mammalian cells plays a major role in balancing anabolic processes such as the synthesis of proteins, lipids, and nucleotides and catabolic processes, such as autophagy in response to environmental cues, such as availability of nutrients and growth factors.
The mTOR pathway or the...
The mTOR pathway or the...
4.5K
01:20The JAK-STAT Signaling Pathway
11.6K
Several cytokine receptors have tightly bound Janus kinase or JAK proteins attached at their cytosolic tail. Small signaling molecules such as cytokines, growth hormones, or prolactins bind to the cytokine receptors and initiate their dimerization. The dimerization brings the cytosolic JAKs together that trans-phosphorylate and activates each other. The activated JAKs now phosphorylate cytosolic tails of the cytokine receptors, which serve as binding sites for adaptor proteins such as SH2...
11.6K
01:19Interactions Between Signaling Pathways
7.1K
Signaling cascades usually lack linearity. Multiple pathways interact and regulate one another, allowing cells to integrate and respond to diverse environmental stimuli.
Convergence and divergence, and cross-talk between signaling pathways
Two distinct signaling pathways can converge on a single functional unit, which may either be a single protein or a complex of proteins. The response is either functionally distinct or synergistic between the two pathways but different from the response...
Convergence and divergence, and cross-talk between signaling pathways
Two distinct signaling pathways can converge on a single functional unit, which may either be a single protein or a complex of proteins. The response is either functionally distinct or synergistic between the two pathways but different from the response...
7.1K
01:20The Retinoblastoma Gene
4.6K
Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
4.6K
03:14Notch Signaling Pathway
6.3K
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...
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...
6.3K