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

Alternative complement pathway activity in sera from patients with sickle cell disease

S M Koethe, J T Casper, G E Rodey

    Clinical and Experimental Immunology
    |January 1, 1976
    PubMed
    Summary

    Sickle cell disease (SCD) patients show reduced cobra venom factor (CoVF) cofactor activity, indicating a deficiency in C3PA convertase, a key component of the alternative complement pathway.

    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

    Alternative donor bone marrow transplant for children with Philadelphia chromosome ALL.

    Bone marrow transplantation·2005
    Same author

    Evaluation of histogenesis of B-lymphocytes in pediatric EBV-related post-transplant lymphoproliferative disorders.

    Bone marrow transplantation·2003
    Same author

    Analysis of risk factors for the development of GVHD after T cell-depleted allogeneic BMT: effect of HLA disparity, ABO incompatibility, and method of T-cell depletion.

    Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation·2002
    Same author

    Protection from lethal murine graft-versus-host disease without compromise of alloengraftment using transgenic donor T cells expressing a thymidine kinase suicide gene.

    Blood·2001
    Same author

    Impact of donor type on outcome of bone marrow transplantation for Wiskott-Aldrich syndrome: collaborative study of the International Bone Marrow Transplant Registry and the National Marrow Donor Program.

    Blood·2001
    Same author

    Engraftment and survival after unrelated-donor bone marrow transplantation: a report from the national marrow donor program.

    Blood·2000

    Area of Science:

    • Immunology
    • Hematology

    Background:

    • The alternative complement pathway is crucial for immune response.
    • Sickle cell disease (SCD) is a genetic blood disorder.
    • Complement system dysregulation may contribute to SCD complications.

    Purpose of the Study:

    • To investigate the alternative complement pathway activity in SCD sera.
    • To determine the levels of C3 proactivator (C3PA) and C3PA convertase in SCD patients.

    Main Methods:

    • Activation of the alternative complement pathway using cobra venom factor (CoVF).
    • Assessing CoVF cofactor activity in SCD and normal sera via indirect lysis assay.
    • Quantifying C3PA levels using immunoelectrophoresis and radial immunodiffusion.

    Main Results:

    Related Experiment Videos

    • SCD sera exhibited significantly reduced CoVF cofactor activity compared to normal sera.
    • C3PA levels were normal or elevated in SCD sera.
    • This suggests a specific deficiency in C3PA convertase activity.

    Conclusions:

    • Sera from sickle cell disease patients demonstrate diminished C3PA convertase activity.
    • This finding may have implications for understanding immune function in SCD.