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Notch Signaling Pathway03:14

Notch Signaling Pathway

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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.
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...
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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
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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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When a ligand binds to a cell-surface receptor, the receptor's intracellular domain changes shape, which may either activate its enzyme function or allow its binding to other molecules. The initial signal is amplified by most signal transduction pathways. This means that a single ligand molecule can activate multiple molecules of a downstream target. Proteins that relay a signal are most commonly phosphorylated at one or more sites, activating or inactivating the protein. Kinases catalyze...
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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...
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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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Stimulation of Notch Signaling in Mouse Osteoclast Precursors
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Signaling pathways affected by mutations causing osteogenesis imperfecta.

Julia Etich1, Mirko Rehberg2, Beate Eckes3

  • 1Dr. Rolf M. Schwiete Research Unit for Osteoarthritis, Orthopedic University Hospital Friedrichsheim gGmbH, Frankfurt/Main, 60528, Germany.

Cellular Signalling
|September 27, 2020
PubMed
Summary
This summary is machine-generated.

Osteogenesis imperfecta (OI) is a bone disorder causing fragility and deformity. This review explores signaling pathways linked to OI genes, impacting bone remodeling and extracellular matrix integrity.

Keywords:
RANK/RANKLTGFbetaWNTintegrinsosteogenesis imperfectasignal transductionunfolded protein response

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Area of Science:

  • Biochemistry
  • Genetics
  • Cell Biology

Background:

  • Osteogenesis imperfecta (OI) is a heterogeneous connective tissue disorder marked by bone fragility and skeletal deformities.
  • Bone integrity relies on extracellular matrix (ECM) remodeling, regulated by osteoclasts and osteoblasts.
  • Over 20 OI forms exist, caused by mutations in collagen type I genes or genes affecting collagen processing, osteoblast function, or mineralization.

Purpose of the Study:

  • To provide an overview of signaling pathways associated with known OI-causing genes.
  • To discuss the impact of these genes on signal transduction in the context of OI.

Main Methods:

  • Literature review of signaling pathways implicated in Osteogenesis Imperfecta.
  • Analysis of gene functions related to collagen processing, osteoblast activity, and bone mineralization.
  • Examination of intracellular homeostasis and ECM structure in non-classical OI forms.

Main Results:

  • Identified key signaling pathways including WNT, RANK/RANKL, TGFβ, MAPK, and integrin-mediated signaling.
  • Highlighted the role of the unfolded protein response in OI pathogenesis.
  • Emphasized altered ECM structure and disturbed intracellular homeostasis in non-classical OI forms.

Conclusions:

  • Understanding these signaling pathways is crucial for elucidating OI mechanisms.
  • These pathways influence bone remodeling, ECM integrity, and cellular homeostasis in OI.
  • Further research into these signaling networks may reveal therapeutic targets for Osteogenesis Imperfecta.