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Related Concept Videos

Activation and Inactivation of G Proteins01:22

Activation and Inactivation of G Proteins

Heterotrimeric G proteins are guanine nucleotide-binding proteins. As the name suggests, heterotrimeric G proteins are composed of three subunits: alpha, beta, and gamma. They remain GDP-bound or GTP-bound inside the cells and switch between inactive/active states. The Gα subunit possesses the nucleotide-binding pocket that binds guanine nucleotides and switches between GDP or GTP-bound states. In contrast, the Gꞵ and Gγ subunits are always bound together with high affinity and are together...
GTPases and their Regulation02:14

GTPases and their Regulation

Guanine nucleotide-binding proteins (G-proteins), also known as GTPases, are a superfamily of proteins that regulate many cellular processes, such as cell signaling, vesicular transport, and the regulation of cell shape and motility. Mutation or dysfunction of these proteins can lead to disease. There are around 40,000 known G-proteins that can broadly be classified into two groups ‒  small G-proteins consisting of a single domain and large multi-domain G-proteins.
Large G-proteins, also known...
GTPases and their Regulation02:14

GTPases and their Regulation

Guanine nucleotide-binding proteins (G-proteins), also known as GTPases, are a superfamily of proteins that regulate many cellular processes, such as cell signaling, vesicular transport, and the regulation of cell shape and motility. Mutation or dysfunction of these proteins can lead to disease. There are around 40,000 known G-proteins that can broadly be classified into two groups ‒  small G-proteins consisting of a single domain and large multi-domain G-proteins.
Large G-proteins, also known...
Small GTPases - Ras and Rho01:24

Small GTPases - Ras and Rho

Ras and Rho are small monomeric GTPases that act downstream of receptor tyrosine kinase (RTK) and regulate various cellular processes. These GTPases switch between active and inactive states by binding to guanine nucleotides.
Three regulatory proteins control their activity:
TGF - β Signaling Pathway01:16

TGF - β Signaling Pathway

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 are of three kinds RI, RII, and RIII. The RI...
Notch Signaling Pathway03:14

Notch Signaling Pathway

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 until 1985...

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Related Experiment Video

Updated: May 30, 2026

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis
07:26

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis

Published on: April 1, 2022

Gli proteins in development and disease.

Chi-Chung Hui1, Stephane Angers

  • 1Program in Developmental and Stem Cell Biology, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada. cchui@sickkids.ca

Annual Review of Cell and Developmental Biology
|August 2, 2011
PubMed
Summary

Gli proteins are key transcription factors regulated by Hedgehog signaling. Their dysregulation contributes to congenital malformations and cancer, involving complex posttranslational modifications and signaling pathways.

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Last Updated: May 30, 2026

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Light-mediated Reversible Modulation of the Mitogen-activated Protein Kinase Pathway during Cell Differentiation and Xenopus Embryonic Development
09:32

Light-mediated Reversible Modulation of the Mitogen-activated Protein Kinase Pathway during Cell Differentiation and Xenopus Embryonic Development

Published on: June 15, 2017

Area of Science:

  • Molecular Biology
  • Developmental Biology
  • Cancer Biology

Background:

  • Gli proteins are transcription factors crucial for Hedgehog signaling, a pathway implicated in development and disease.
  • Mutations and aberrant regulation of Gli proteins are linked to human congenital malformations and tumorigenesis.
  • Hedgehog signaling controls Gli protein activity through posttranslational modifications and nucleocytoplasmic transport.

Purpose of the Study:

  • To review the intricate regulation of Gli proteins during embryonic development.
  • To explore the mechanisms underlying the abnormal activation of Gli proteins in tumorigenesis.
  • To highlight both canonical Hedgehog-dependent and noncanonical regulatory pathways.

Main Methods:

  • Literature review of genetic studies and molecular mechanisms.
  • Analysis of posttranslational modifications affecting Gli protein activity.
  • Investigation of the role of primary cilia and core regulators like Suppressor of fused and Kif7.

Main Results:

  • Gli protein activity is tightly controlled by Hedgehog signaling via phosphorylation, degradation, and processing.
  • Primary cilia in vertebrate cells are essential for sensing Hedgehog pathway activity and Gli protein activation.
  • Noncanonical mechanisms, independent of Hedgehog signaling, also contribute to Gli protein regulation.

Conclusions:

  • Understanding Gli protein regulation is vital for deciphering developmental processes and disease pathogenesis.
  • Aberrant Gli activation through various mechanisms drives tumorigenesis.
  • Further research into both canonical and noncanonical pathways is necessary for therapeutic strategies.