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Related Concept Videos

The Ras Gene02:38

The Ras Gene

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The Ras-gene-encoded proteins are regulators of signaling pathways controlling cell proliferation, differentiation, or cell survival. The Ras-gene family in humans constitutes three primary members—the HRas, NRas, and KRas. These genes code for four functionally distinct yet closely related proteins—the HRas, NRas, KRas4A, and KRas4B. The involvement of mutant Ras genes in human cancer was first discovered in 1982 and is among the most common causes of human tumorigenesis.
Ras is a...
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Small GTPases - Ras and Rho01:24

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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.
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MAPK Signaling Cascades01:07

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Mitogen-activated protein kinase, or MAPK pathway, activates three sequential kinases to regulate cellular responses such as proliferation, differentiation, survival, and apoptosis. The canonical MAPK pathway starts with a mitogen or growth factor binding to an RTK. The activated RTKs stimulate Ras, which recruits Raf or MAP3 Kinase (MAPKKK), the first kinase of the MAPK signaling cascade. Raf further phosphorylates and activates MEK or MAP2 Kinases (MAPKK), which in turn phosphorylates MAP...
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Abnormal Proliferation02:23

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Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the...
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Inhibition of Cdk Activity02:34

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The orderly progression of the cell cycle depends on the activation of Cdk protein by binding to its cyclin partner. However, the cell cycle must be restricted when undergoing abnormal changes. Most cancers correlate to the deregulated cell cycle, and since Cdks are a central component of the cell cycle, Cdk inhibitors are extensively studied to develop anticancer agents. For instance, cyclin D associates with several Cdks, such as Cdk 4/6, to form an active complex. The cyclin D-Cdk4/6 complex...
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The JAK-STAT Signaling Pathway01:20

The JAK-STAT Signaling Pathway

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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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Characterize Disease-related Mutants of RAF Family Kinases by Using a Set of Practical and Feasible Methods
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MEK inhibitors in RASopathies.

Christina Bergqvist1,2, Pierre Wolkenstein1,2,3,4

  • 1Assistance Publique-Hôpital Paris (AP-HP), Hôpital Henri-Mondor, Service de Dermatologie.

Current Opinion in Oncology
|January 4, 2021
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MEK inhibitors (MEKi) show promise for treating RAS-driven cancers and RASopathies like Neurofibromatosis 1 (NF1). Selumetinib, a MEKi, is FDA-approved for NF1-related plexiform neurofibromas in children.

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Area of Science:

  • Oncology
  • Molecular Biology
  • Genetics

Background:

  • The Ras/Raf/MEK/ERK pathway regulates cell proliferation.
  • Dysregulation of this pathway is implicated in RAS-driven neoplasias and RASopathies.
  • MEK inhibitors (MEKi) have been developed as targeted therapies.

Purpose of the Study:

  • To review the role of MEK inhibitors in treating RASopathies.
  • To highlight the efficacy of MEKi in Neurofibromatosis 1 (NF1).
  • To discuss future directions for MEK inhibition in RASopathies.

Main Methods:

  • Review of early-phase clinical trials involving MEK inhibitors.
  • Analysis of data from the SPRINT trial of selumetinib in pediatric NF1 patients.
  • Evaluation of selumetinib's efficacy in low-grade gliomas (LGGs).

Main Results:

  • Selumetinib demonstrated significant tumor reduction in plexiform neurofibromas (pNF) in NF1 patients.
  • Selumetinib achieved FDA approval for pediatric symptomatic pNF.
  • Positive response rates and progression-free survival were observed in LGG patients treated with selumetinib.

Conclusions:

  • MEK inhibition is a promising and well-tolerated treatment for NF1.
  • The use of targeted agents like MEKi is expected to increase in the NF1 population.
  • Further research is needed for non-NF1 RASopathies, focusing on preclinical models and clinical trial endpoints.