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A critical role for complement in maintenance of self-tolerance
A P Prodeus1, S Goerg, L M Shen
1The Center for Blood Research, Department of Pathology, Harvard Medical School, Boston, Massachusetts 02115, USA.
Immunity
|December 10, 1998
Summary
The complement system is crucial for B cell tolerance, preventing autoimmune diseases like lupus. Complement deficiencies impair self-tolerance, increasing susceptibility to systemic lupus erythematosus.
Area of Science:
- Immunology
- Autoimmunity
- Complement System Biology
Background:
- The complement system plays a role in immune regulation.
- Self-tolerance mechanisms prevent autoimmune diseases.
- Systemic lupus erythematosus (SLE) is an autoimmune disease with complex genetic associations.
Purpose of the Study:
- To investigate the role of complement in maintaining B cell self-tolerance.
- To explore the link between complement deficiency and SLE susceptibility.
Main Methods:
- Utilized an immunoglobulin transgenic model for peripheral tolerance.
- Studied a lupus-like murine model with CD95 (Fas) deficiency.
- Assessed B cell anergy in mice deficient in complement receptors (CD21/CD35) or complement protein C4.
Main Results:
- Self-reactive B cells lacking complement receptors CD21/CD35 or in C4-deficient mice failed to be anergized by self-antigen.
- Combined deficiencies in CD21/CD35 or C4 with CD95 deficiency led to high anti-nuclear antibody titers and severe lupus-like disease.
Conclusions:
- The complement system has a novel role in B cell tolerance.
- Complement deficiency is linked to increased susceptibility to systemic lupus erythematosus.