Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[Human pathologies associated with NF-kappaB defects].

Gilles Courtois1, Alain Israël

  • 1Unité de Biologie Moléculaire de l'Expression Génique,URA 2582 CNRS, Institut Pasteur, 25, rue du Dr Roux, 75724 Paris Cedex 15, France.

Journal De La Societe De Biologie
|September 17, 2004
PubMed
Summary

Nuclear factor-kappa B (NF-kappaB) regulates immune and inflammatory responses. Its activation involves the IkappaB kinase (IKK) complex, and defects in this pathway cause human diseases, highlighting IKK

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Author Correction: TNF-mediated inflammatory skin disease in mice with epidermis-specific deletion of IKK2.

Nature·2025
Same author

Incontinentia pigmenti underlies thymic dysplasia, autoantibodies to type I IFNs, and viral diseases.

The Journal of experimental medicine·2024
Same author

NLRP7 Enhances Choriocarcinoma Cell Survival and Camouflage in an Inflammasome Independent Pathway.

Cells·2023
Same author

The necroptosis-inducing pseudokinase mixed lineage kinase domain-like regulates the adipogenic differentiation of pre-adipocytes.

iScience·2022
Same author

Binaural Signal Processing in Hearing Aids.

Seminars in hearing·2021
Same author

Perception of Auditory Distance in Normal-Hearing and Moderate-to-Profound Hearing-Impaired Listeners.

Trends in hearing·2019

Area of Science:

  • Molecular Biology
  • Immunology
  • Cellular Signaling

Context:

  • NF-kappaB transcription factors are crucial regulators of immune, inflammatory, and anti-apoptotic responses.
  • NF-kappaB dimers are held in the cytoplasm by IkappaB inhibitors, masking nuclear localization signals.
  • IKK complex integrates signals for NF-kappaB activation, involving IKKalpha, IKKbeta, and NEMO/IKKgamma subunits.

Purpose:

  • To elucidate the precise molecular mechanisms of IKK complex activation.
  • To investigate the role of IKK complex in human pathologies.
  • To explore the involvement of non-degradation-linked ubiquitination in NF-kappaB activation.

Summary:

  • Cellular stimulation leads to IkappaB phosphorylation by IKK, followed by ubiquitination and proteasomal degradation, releasing NF-kappaB for nuclear translocation and gene activation.

Related Experiment Videos

  • While IkappaB degradation is understood, the activation mechanisms of the high-molecular-weight IKK complex require further elucidation.
  • Four human pathologies linked to IKK complex dysfunction are described, including mutations in NEMO, IkappaBalpha, and a deubiquitinase interacting with NEMO.

    • Impact:

    • The IKK complex's central role is underscored by its involvement in diverse human pathologies.
    • Findings strengthen the hypothesis of non-degradation-linked ubiquitination's role in NF-kappaB activation.
    • Understanding these pathways is critical for developing therapeutic strategies for immune and inflammatory diseases.