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Lupus in the Fas lane?
1New England Medical Center and Tufts University School of Medicine, Boston, MA, USA.
Journal of the Royal College of Physicians of London
|November 1, 1995
Summary
The Fas protein is crucial for lymphocyte apoptosis. Defective Fas in lupus models leads to autoimmune disease, suggesting Fas
Area of Science:
- Immunology
- Molecular Biology
- Autoimmune Diseases
Background:
- Fas is a 43 kDa glycoprotein molecule that induces apoptosis in B and T lymphocytes.
- The lymphoproliferation (lpr) mutation in murine models of systemic lupus erythematosus (SLE) causes defective Fas protein gene transcription.
- MRL/Ipr-Ipr mice with the homozygous recessive Ipr mutation develop a severe, genetically predetermined autoimmune syndrome.
Purpose of the Study:
- To review the importance of Fas in the pathogenesis of autoimmune diseases, particularly SLE.
- To discuss the role of Fas in lymphocyte apoptosis and its implications in SLE.
Main Methods:
- Review of existing literature on Fas, apoptosis, and systemic lupus erythematosus.
- Analysis of data from murine MRL/Ipr-Ipr models of SLE.
- Examination of human SLE patient data regarding soluble Fas receptor levels.
Main Results:
- The lpr mutation in mice leads to defective Fas transcription, resulting in autoimmune syndrome with high autoantibody levels and glomerulonephritis.
- Elevated soluble Fas receptor levels are observed in approximately 60% of human SLE patients.
- Abnormal soluble Fas receptor presence may protect lymphocytes from undergoing apoptosis.
Conclusions:
- Fas-mediated apoptosis plays a critical role in preventing autoimmune diseases like SLE.
- Defects in the Fas pathway contribute to the pathogenesis of SLE.
- Soluble Fas receptor may serve as a biomarker and therapeutic target in SLE.