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Related Concept Videos

NF-κB-dependent Signaling Pathway02:26

NF-κB-dependent Signaling Pathway

The transcription factor NF-κB was discovered in 1986 in the lab of Nobel laureate Professor David Baltimore, for its interaction with the immunoglobulin light chain enhancer in B-cells. After more than three decades of study, it is now evident that NF-κB regulates the expression of over 100 genes. Most of these genes play an essential role in the innate and adaptive immune responses as well as the inflammatory responses of animals.
NF-κB-dependent Signaling Mechanism
The heterodimer of NF-κB...
NF-kB-dependent Signaling Pathway02:26

NF-kB-dependent Signaling Pathway

The transcription factor NF-κB was discovered in 1986 in the lab of Nobel laureate Professor David Baltimore, for its interaction with the immunoglobulin light chain enhancer in B-cells. After more than three decades of study, it is now evident that NF-κB regulates the expression of over 100 genes. Most of these genes play an essential role in the innate and adaptive immune responses as well as the inflammatory responses of animals.
NF-κB-dependent Signaling Mechanism
The heterodimer of NF-κB...
Enzyme-linked Receptors01:00

Enzyme-linked Receptors

Enzyme-linked receptors are proteins that act as both receptor and enzyme, activating multiple intracellular signals. This is a large group of receptors that include the receptor tyrosine kinase (RTK) family. Many growth factors and hormones bind to and activate the RTKs.
Neurotrophin (NT) receptors are a family of RTKs, including trkA, trkB, and trkC (tropomyosin-related kinase) receptors. TrkA is specific for nerve growth factor (NGF), neurotrophin-6, and neurotrophin-7. TrkB binds...
The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
Co-activators and Co-repressors02:04

Co-activators and Co-repressors

Gene transcription is regulated by the synergistic action of several proteins that form a complex at a gene regulatory site. This is observed in eukaryotes, where the regulation of gene expression is a complex process. Regulatory proteins in eukaryotes can broadly be classified into two types – regulators that bind directly to specific DNA sequences and co-regulators that associate with regulatory proteins but cannot directly bind to the DNA. These co-regulators are further divided into...
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...

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Related Experiment Video

Updated: May 21, 2026

Protocol for the Differentiation of Human Induced Pluripotent Stem Cells into Mixed Cultures of Neurons and Glia for Neurotoxicity Testing
09:02

Protocol for the Differentiation of Human Induced Pluripotent Stem Cells into Mixed Cultures of Neurons and Glia for Neurotoxicity Testing

Published on: June 9, 2017

Impaired NF-κB/Nrf2 Crosstalk in Rett Syndrome.

Valeria Cordone1, Andrea Vallese2, Andrea Bianchi1

  • 1Department of Life, Health and Environmental Sciences, University of L'Aquila, L'Aquila, Italy.

FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology
|May 20, 2026
PubMed
Summary
This summary is machine-generated.

Rett syndrome (RTT) involves an OxInflammatory state due to dysfunctional NF-κB/Nrf2 signaling, leading to inflammation and impaired antioxidant defense. Combined Nrf2 activation and NF-κB inhibition partially restored cellular balance in RTT fibroblasts.

Related Experiment Videos

Last Updated: May 21, 2026

Protocol for the Differentiation of Human Induced Pluripotent Stem Cells into Mixed Cultures of Neurons and Glia for Neurotoxicity Testing
09:02

Protocol for the Differentiation of Human Induced Pluripotent Stem Cells into Mixed Cultures of Neurons and Glia for Neurotoxicity Testing

Published on: June 9, 2017

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Cell Biology

Background:

  • Rett syndrome (RTT) is a neurodevelopmental disorder linked to MECP2 mutations, characterized by mitochondrial dysfunction, oxidative stress, and inflammation.
  • The OxInflammatory state in RTT suggests disrupted redox-inflammatory pathways, involving NF-κB (nuclear factor kappa-light-chain-enhancer of activated B cells) and Nrf2 (Nuclear factor erythroid 2-related factor 2) signaling.
  • NF-κB and Nrf2 crosstalk is crucial for balancing inflammation and antioxidant responses.

Purpose of the Study:

  • To investigate the functional status of the NF-κB/Nrf2 signaling crosstalk in primary dermal fibroblasts from RTT patients and healthy controls.
  • To explore the mechanisms underlying the dysregulated signaling axis in RTT.
  • To assess the potential of combined NF-κB inhibition and Nrf2 activation as a therapeutic strategy.

Main Methods:

  • Primary dermal fibroblasts from RTT patients and controls were analyzed under basal and lipopolysaccharide (LPS)-stimulated conditions.
  • NF-κB and Nrf2 pathway activation, acetylation dynamics (CBP/p300, SIRT1), and downstream gene expression were assessed.
  • Cells were treated with sulforaphane (SFN), an Nrf2 activator, and BAY-117082, an NF-κB inhibitor, to study crosstalk modulation.

Main Results:

  • RTT fibroblasts exhibited basal NF-κB activation and impaired Nrf2 response, indicating a pro-inflammatory and imbalanced state.
  • LPS stimulation failed to elicit coordinated NF-κB and Nrf2 activation in RTT cells, unlike controls.
  • Combined SFN and BAY-117082 treatment in RTT fibroblasts reduced pro-inflammatory cytokines and enhanced antioxidant gene expression (HMOX1).

Conclusions:

  • RTT is characterized by a dysfunctional NF-κB/Nrf2 regulatory axis with constitutive inflammation and inadequate antioxidant compensation.
  • Altered acetylation dynamics, with increased CBP/p300 and normal SIRT1, may contribute to persistent NF-κB activity in RTT.
  • Targeting the NF-κB/Nrf2 axis, particularly through combined inhibition and activation, shows promise for mitigating the OxInflammatory state in RTT.