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

Deficiencies of the complement system in man

N K Day, R A Good

    Birth Defects Original Article Series
    |January 1, 1975
    PubMed
    Summary
    This summary is machine-generated.

    Complement deficiencies impact immune function, leading to infections and autoimmune diseases. Reconstitution therapies, like bone marrow or kidney transplants, can restore complement function in deficient patients.

    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

    Identification of a novel allele HLA-DQB1*0622.

    Tissue antigens·2007
    Same author

    Characterization of a novel allele HLA-B*3549.

    Tissue antigens·2005
    Same author

    A retroviral-derived immunosuppressive peptide activates mitogen-activated protein kinases.

    Journal of immunology (Baltimore, Md. : 1950)·2001
    Same author

    Immunologic reconstitution following bone marrow transplantation for X-linked hyper IgM syndrome.

    Clinical immunology (Orlando, Fla.)·2001
    Same author

    Involvement of a herbimycin A-sensitive protein tyrosine kinase in extracellular action of HIV-1 Nef.

    Immunology letters·2001
    Same author

    Monocytes are target cells for IL-10 induction by HIV-1 Nef protein.

    Cytokine·2000
    Same journal

    A case of short-rib syndrome without polydactyly in a stillborn: a new type?

    Birth defects original article series·1996
    Same journal

    Hand malformations in the aborted embryo: an important source of genetic information.

    Birth defects original article series·1996
    Same journal

    Colophon: on doing morphology.

    Birth defects original article series·1996
    Same journal

    Congenital eye malformations: a descriptive epidemiologic study in about one million newborns in Italy.

    Birth defects original article series·1996
    Same journal

    CHARGE association in a neonate exposed in utero to carbon monoxide.

    Birth defects original article series·1996
    Same journal

    Fetal valproate embryopathy in twins: genetic modification of the response to a teratogen.

    Birth defects original article series·1996
    See all related articles

    Area of Science:

    • Immunology
    • Human Genetics
    • Clinical Medicine

    Background:

    • Deficiencies in complement system components can lead to significant health issues.
    • Specific complement deficiencies are linked to increased susceptibility to infections and autoimmune disorders.
    • The complement system plays a crucial role in innate and adaptive immunity.

    Purpose of the Study:

    • To describe complement component deficiencies in humans.
    • To investigate the outcomes of reconstituting complement function in deficient patients.
    • To explore the relationship between complement deficiencies and associated clinical manifestations.

    Main Methods:

    • Case descriptions of patients with complement deficiencies.
    • Monitoring of complement system reconstitution after transplantation (bone marrow and kidney).

    Related Experiment Videos

  • Observation of clinical outcomes and laboratory parameters (e.g., C3 levels) following interventions.

    • Main Results:

    • Complement component Clq was successfully reconstituted in severe combined immunodeficiency patients post-bone marrow transplant.
    • Clq deficiency is associated with vascular disease and recurrent upper respiratory infections.
    • Complement reconstitution was observed in a Clr-deficient patient after kidney transplantation.
    • A C2-deficient patient with systemic lupus erythematosus showed elevated C3 levels.
    • Blood transfusion, providing C2, resulted in significant in vivo complement activation in a C2-deficient patient.

    Conclusions:

    • Complement deficiencies present with diverse clinical phenotypes, including immunodeficiency and autoimmune conditions.
    • Therapeutic interventions such as transplantation can restore complement function.
    • Understanding complement pathways is vital for managing associated diseases and developing effective treatments.