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Quantitative PCR-based Assay to Measure Sonic Hedgehog Signaling in Cellular Model of Ciliogenesis
Published on: January 31, 2025
Smoothened transduces Hedgehog signal by forming a complex with Evc/Evc2
Cuiping Yang1, Wenlin Chen, Yongbin Chen
1Key Laboratory of Animal Models and Human Disease Mechanisms of Chinese Academy of Sciences and Yunnan Province, Kunming Institute of Zoology, Chinese Academy of Sciences, 32 Jiaochang Donglu, Kunming, Yunnan 650223, China.
Cell Research
|September 19, 2012
Summary
The Hedgehog (Hh) pathway uses ciliary proteins Evc and Evc2 to transmit signals from Smoothened (Smo) to Gli transcription factors, crucial for development and tissue repair.
Area of Science:
- Cell Biology
- Developmental Biology
- Genetics
Background:
- Hedgehog (Hh) signaling is vital for embryonic development and tissue homeostasis.
- Smoothened (Smo) transduces the Hh signal, but downstream mechanisms in mammals remain unclear.
- Ellis-van Creveld syndrome is linked to mutations in Evc and Evc2.
Purpose of the Study:
- To elucidate the role of ciliary proteins Evc and Evc2 in mammalian Hh signal transduction downstream of Smo.
- To investigate the interaction between Evc/Evc2, Smo, and Gli transcription factors.
- To understand the mechanism of Hh pathway activation in primary cilia.
Main Methods:
- Utilized genetic knockout models (Kif3a-/-) and cell-specific mutations.
- Investigated Smo phosphorylation and ciliary localization.
- Assessed Gli transcriptional activity and protein interactions.
- Examined the impact of Evc/Evc2 loss on Hh pathway activation.
Main Results:
- Evc and Evc2 function downstream of Smo to mediate Hh signal transduction.
- Loss of Evc/Evc2 impairs Hh pathway activation, particularly with constitutively active Smo.
- Evc/Evc2 act upstream of Sufu in promoting Gli activation.
- Hh signaling induces Evc/Evc2 binding to phosphorylated Smo within primary cilia.
Conclusions:
- Evc and Evc2 are essential ciliary components for transducing Hh signals from Smo to Gli proteins.
- Hh activation involves Smo phosphorylation, recruiting Evc/Evc2 to antagonize Sufu and activate Gli in primary cilia.
- This mechanism is critical for Hh pathway function and understanding Ellis-van Creveld syndrome.
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