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

Complement and IgG subclasses in agammaglobulinemic patients

A S Grumach1, M Kirschfink

  • 1Department of Pediatrics, Faculty of Medicine, University of São Paulo, Brazil.

Experimental and Clinical Immunogenetics
|January 1, 1995
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

The Chronic Angioedema Registry (CARE): Rationale, Methods and Implementation.

Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology·2024
Same author

Arerugi = [Allergy]·2023
Same author

Arerugi = [Allergy]·2023
Same author

Arerugi = [Allergy]·2023
Same author

Variability of disease activity in patients with hereditary angioedema type 1/2: longitudinal data from the Icatibant Outcome Survey.

Journal of the European Academy of Dermatology and Venereology : JEADV·2021
Same author

Uncommon Signs Associated With Hereditary Angioedema With Normal C1 Inhibitor.

Journal of investigational allergology & clinical immunology·2020

Agammaglobulinemia patients with recurrent bacterial infections show reduced IgG subclasses and activated complement systems. Sensitive analysis reveals varied immunoglobulin synthesis, potentially explaining diverse clinical symptoms and increased infection susceptibility.

Area of Science:

  • Immunology
  • Clinical Medicine

Background:

  • Agammaglobulinemia is characterized by recurrent bacterial infections.
  • Patients often exhibit significantly reduced levels of immunoglobulins, including IgG subclasses, IgM, and IgA.
  • The role of complement system activation in these patients requires further elucidation.

Purpose of the Study:

  • To analyze complement and IgG subclass levels in agammaglobulinemic patients.
  • To investigate the relationship between immunoglobulin levels, complement activation, and recurrent infections.
  • To understand the heterogeneity in clinical presentation among agammaglobulinemic patients.

Main Methods:

  • Functional assays for classical (CH50) and alternative (APH50) complement pathways.
  • Quantification of complement proteins (C3, C4, factor B) and regulators (C1-inhibitor, factors H and I).

Related Experiment Videos

  • Measurement of C3-derived split product (C3dg/C3d) and IgG subclasses using sensitive ELISA.

    • Main Results:

    • Markedly reduced IgG subclass levels were observed in all patients.
    • Pronounced complement system activation was evident, indicated by reduced hemolytic function and elevated C3dg/C3d levels.
    • Heterozygous C4 deficiency was considered in some patients due to depressed C4A and C4B levels.

    Conclusions:

    • Agammaglobulinemic patients exhibit significant variability in immunoglobulin synthesis, detectable by sensitive methods.
    • The activated and depleted complement system reflects increased susceptibility to bacterial infections.
    • Varied immunoglobulin synthesis may contribute to the heterogeneous clinical symptoms observed in these patients.