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

Inherited deficiencies of complement and complement-related proteins.

L F Fries, J J O'Shea, M M Frank

    Clinical Immunology and Immunopathology
    |July 1, 1986
    PubMed
    Summary

    Deficiencies in the complement system, crucial for host defense, can lead to autoimmune diseases or increased infection risk. Management requires careful diagnosis and treatment of associated conditions.

    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

    Cholesterol 25-hydroxylase is a metabolic switch to constrain T cell-mediated inflammation in the skin.

    Science immunology·2021
    Same author

    Complement activation.

    Immunology today·2014
    Same author

    Altered balance between Th17 and Th1 cells at mucosal sites predicts AIDS progression in simian immunodeficiency virus-infected macaques.

    Mucosal immunology·2008
    Same author

    Key molecules involved in receptor-mediated lymphocyte activation.

    Current protocols in immunology·2008
    Same author

    Wild-type adenoviruses from groups A-F evoke unique innate immune responses, of which HAd3 and SAd23 are partially complement dependent.

    Gene therapy·2008
    Same author

    Recombinant adenovirus vectors activate the alternative complement pathway, leading to the binding of human complement protein C3 independent of anti-ad antibodies.

    Molecular therapy : the journal of the American Society of Gene Therapy·2004

    Area of Science:

    • Immunology
    • Genetics

    Background:

    • The complement system is vital for host defense, immune complex processing, and immunoregulation.
    • Genetic deficiencies in complement proteins, inhibitors, or receptors are linked to clinical issues.
    • These deficiencies manifest primarily as autoimmune diseases or heightened susceptibility to infections.

    Purpose of the Study:

    • To review the clinical significance of complement system deficiencies.
    • To differentiate clinical outcomes based on complement pathway involvement.
    • To discuss current management strategies for complement-related disorders.

    Main Methods:

    • Literature review of complement deficiency states.
    • Analysis of clinical manifestations associated with specific complement defects.
    • Examination of diagnostic and therapeutic approaches.

    Main Results:

    • Classical pathway and CR1 deficiencies are often linked to autoimmune disorders.
    • Alternative pathway, terminal component, and CR3 defects are associated with increased risk of pyogenic bacterial infections.
    • Clinical presentations can overlap, and not all deficiencies strictly adhere to these associations.

    Conclusions:

    • Complement deficiencies present significant health risks, including autoimmunity and infections.
    • While some patterns exist, a broad diagnostic suspicion is necessary for all complement defects.
    • Targeted therapy is available for C1-inhibitor deficiency; others require comprehensive management of complications.

    Related Experiment Videos