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Related Concept Videos

Hedgehog Signaling Pathway02:33

Hedgehog Signaling Pathway

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...
Hedgehog Signaling Pathway02:33

Hedgehog Signaling Pathway

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...
Microtubules in Signaling01:22

Microtubules in Signaling

The primary cilium, made up of microtubules, acts as antennae on the cell surfaces for relaying external stimuli into the cells. These fine hair-like structures are present, generally one per cell. These are non-motile cilia in a 9+0 microtubules arrangement, where the central pair of microtubules are absent. The primary cilia arise from the basal body embedded in the cell membrane. Intraflagellar transport (IFT) carries requisite proteins from the cytoplasm to the cilium because the primary...
Mechanism of Ciliary Motion01:05

Mechanism of Ciliary Motion

The ciliary structures were first seen in 1647 by Antonie Leeuwenhoek while observing the protozoans. In lower organisms, these appendages are responsible for cell movement, while in higher organisms, these appendages help in the movement of the extracellular fluids within the body cavities.
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...

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Quantitative PCR-based Assay to Measure Sonic Hedgehog Signaling in Cellular Model of Ciliogenesis
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Quantitative PCR-based Assay to Measure Sonic Hedgehog Signaling in Cellular Model of Ciliogenesis

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Hedgehog trafficking, cilia and brain functions.

Martial Ruat1, Hermine Roudaut, Julien Ferent

  • 1CNRS, UPR-3294, Laboratoire de Neurobiologie et Développement, Institut de Neurobiologie Alfred Fessard IFR2118, Signal Transduction and Developmental Neuropharmacology Team, 1 avenue de la Terrasse, F-91198 Gif-sur-Yvette, France. ruat@inaf.cnrs-gif.fr

Differentiation; Research in Biological Diversity
|December 16, 2011
PubMed
Summary

The primary cilium is crucial for Sonic Hedgehog (Shh) signaling, impacting neural tube development and stem cell regulation. Understanding Shh pathway mechanisms in cilia is vital for treating related human diseases and cancers.

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Using Primary Neurosphere Cultures to Study Primary Cilia
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Quantitative PCR-based Assay to Measure Sonic Hedgehog Signaling in Cellular Model of Ciliogenesis
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Efficient and Cost Effective Electroporation Method to Study Primary Cilium-Dependent Signaling Pathways in the Granule Cell Precursor
04:06

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Using Primary Neurosphere Cultures to Study Primary Cilia
08:14

Using Primary Neurosphere Cultures to Study Primary Cilia

Published on: April 14, 2017

Area of Science:

  • Cell Biology
  • Developmental Biology
  • Molecular Biology

Background:

  • The primary cilium acts as a key signaling hub for Sonic Hedgehog (Shh) pathway.
  • Shh signaling via primary cilia is critical for ventral neural tube patterning and adult stem cell regulation.
  • Defects in primary cilia organization are linked to human diseases due to impaired Shh signaling.

Purpose of the Study:

  • To investigate the role of the primary cilium in Sonic Hedgehog (Shh) signal transduction.
  • To understand the mechanism of action of Shh pathway inhibitors in receptor complex trafficking within primary cilia.
  • To explore the connection between primary cilia defects, Shh signaling, and human diseases.

Main Methods:

  • Genetic studies to elucidate Shh signaling at the primary cilium level.
  • Analysis of Shh receptor complex trafficking in response to pathway inhibitors.
  • Investigating the impact of primary cilia organization on Shh pathway activity.

Main Results:

  • Primary cilia are essential for Shh signal transduction.
  • Shh signaling in primary cilia regulates neural tube development and adult stem cells.
  • Defective primary cilia organization can lead to impaired Shh signaling and disease.

Conclusions:

  • The primary cilium is a critical regulator of Shh signaling with implications for development and disease.
  • Targeting Shh signaling at the primary cilium offers therapeutic potential for cancers and other conditions.
  • Further research into Shh pathway mechanisms in primary cilia is warranted for clinical applications.