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

Nephritogenic lambda light chain dimer: a unique human miniautoantibody against complement factor H.

T S Jokiranta1, A Solomon, M K Pangburn

  • 1Complement Research Unit, Department of Bacteriology, Haartman Institute/HD Diagnostics, Helsinki, Finland. sakari.jokiranta@helsinki.fi

Journal of Immunology (Baltimore, Md. : 1950)
|October 8, 1999
PubMed
Summary
This summary is machine-generated.

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

A hypothetical intervention on the use of hearing aids for the risk of dementia in people with hearing loss in UK Biobank.

American journal of epidemiology·2024
Same author

Daytime Sleepiness, Apnea, Neuroimaging Correlates and Cortisol Dysregulation in a Memory Clinic Cohort.

The journal of prevention of Alzheimer's disease·2024
Same author

Evaluation of Clinical Meaningfulness of Fortasyn Connect in Terms of "Time Saved".

The journal of prevention of Alzheimer's disease·2024
Same author

Thermally stable Peltier controlled vacuum chamber for electrical transport measurements.

The Review of scientific instruments·2024
Same author

Validation, Deployment, and Real-World Implementation of a Modular Toolbox for Alzheimer's Disease Detection and Dementia Risk Reduction: The AD-RIDDLE Project.

The journal of prevention of Alzheimer's disease·2024
Same author

Multimodal Precision Prevention - A New Direction in Alzheimer's Disease.

The journal of prevention of Alzheimer's disease·2023
Same journal

Complement C3 deficiency increases the effector and cytotoxic functions of NK cells and suppresses tumor growth.

Journal of immunology (Baltimore, Md. : 1950)·2026
Same journal

Increased Nur77 is disconnected from TCR affinity in insulin-specific Tregs.

Journal of immunology (Baltimore, Md. : 1950)·2026
Same journal

FTR85 negatively regulates type I IFN antiviral signaling pathway by promoting K48-linked polyubiquitination of IRF3.

Journal of immunology (Baltimore, Md. : 1950)·2026
Same journal

An MR1-specific nanobody capable of blocking MR1T cell activation.

Journal of immunology (Baltimore, Md. : 1950)·2026
Same journal

TGF-β controls developmental fate and functional identity of thymic γδ T cells.

Journal of immunology (Baltimore, Md. : 1950)·2026
Same journal

Distinguishing Th17 cells as a unique subset.

Journal of immunology (Baltimore, Md. : 1950)·2026
See all related articles

A unique lambda light chain dimer, protein LOI, was found to activate the alternative pathway of complement. This miniautoantibody blocks factor H, leading to uncontrolled complement activation and severe glomerulonephritis.

Area of Science:

  • Immunology
  • Nephrology
  • Structural Biology

Background:

  • Membranoproliferative glomerulonephritis is a severe kidney disease.
  • The alternative pathway of complement (AP) plays a role in immune responses and inflammation.
  • Factor H is a key regulator of the AP.

Observation:

  • A unique monoclonal Ig lambda light chain dimer (protein LOI) was isolated from a patient with hypocomplementemic membranoproliferative glomerulonephritis.
  • Protein LOI efficiently activated the AP in vitro.
  • LOI bound to factor H, inhibiting its regulatory function.

Findings:

  • The lambda light chain dimer LOI binds to domain 3 of factor H, blocking its interaction with C3b.
  • This blockade leads to uncontrolled AP activation.

Related Experiment Videos

  • Structural analysis revealed an unusual binding site on LOI that complements factor H domain 3.

    • Implications:

    • The lambda light chain dimer LOI acts as a pathogenic miniautoantibody.
    • LOI's mechanism of action provides insight into the pathogenesis of membranoproliferative glomerulonephritis.
    • This discovery opens new avenues for understanding and potentially treating complement-mediated kidney diseases.