These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Epstein-Barr virus transformed B cell lines derived from patients with systemic lupus erythematosus produce a nephritic factor of the classical complement pathway.
    Author: Hiramatsu M, Tsokos GC.
    Journal: Clin Immunol Immunopathol; 1988 Jan; 46(1):91-9. PubMed ID: 2826058.
    Abstract:
    Nephritic factor of the classical complement pathway (C4NeF) is an IgG antibody which stabilizes the C3 convertase (C4b2a) and has been detected in sera from patients with systemic lupus erythematosus (SLE) and acute postinfectious glomerulonephritis. In order to study the production of nephritic factor (NeF), mononuclear cells were isolated from the peripheral blood of patients with SLE and infected with Epstein-Barr virus (EBV) to establish active B lymphocyte cell lines. Supernatants from 15 established B cell lines, as well as from 10 B cell lines established from normal individuals, were investigated for their ability to conserve the classical and the alternative pathway C3 convertases as assessed by EAC3bBb and EAC14b2a stabilizing assays. Supernatants from 2 of 15 B cell lines from patients with SLE, but none from normal individuals, stabilized the classical C3 convertase without having any effect on the alternative pathway C3 convertase. Using anti-human Ig affinity chromatography, we showed that C4NeF activity resided in the IgG fraction; the IgG fraction containing C4NeF activity bound to the C4b2a complex, but not to C4b alone. On gel electrophoresis, following reduction, the heavy chains were slightly heavier than the heavy chains of normal IgG. We were able to isolate C4NeF from the sera of the 2 patients with SLE from whom the positive supernatants were derived, but were unable to detect any C4NeF activity in the sera of the other 13 patients and the 10 normal individuals. Serum and B cell line supernatant-derived C4NeF exhibited comparable characteristics. We conclude that C4NeF produced in vitro by EBV-transformed B cell lines derived from patients with SLE is functionally similar to the conventional C4NeF in serum. These studies confirm the production of autoantibodies by B cells with the ability to stabilize the classical pathway C3 convertase in certain patients with SLE; stabilization of the C4b2a enzyme in these patients is an apparent mechanism for the development of hypocomplementemia. Finally, preparation of homogeneous C4NeF in vitro should improve our understanding of the role of autoantibodies in complement metabolic disturbances in autoimmune diseases.
    [Abstract] [Full Text] [Related] [New Search]