Lupus is a chronic autoimmune inflammatory disease with complex clinical manifestations. In humans, lupus, also known as systemic lupus erythematosus (SLE), affects between 40 and 250 individuals, mostly females, in each 100 000 of the population. There are also a number of murine models of lupus widely used in studies of the genetics, immunopathology, and treatment of lupus. Human patients and murine models of lupus manifest a wide range of immunological abnormalities. The most pervasive of these are: (1) the ability to produce pathogenic autoantibodies; (2) lack of T- and B-lymphocyte regulation; and (3) defective clearance of autoantigens and immune complexes. This article briefly reviews immunological abnormalities and disease mechanisms characteristic of lupus autoimmunity and highlight recent studies on the use of gene therapy to target these abnormalities.
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RA Mageed is supported by grants from Arthritis Research Campaign, UK. The studies of GJ Prud'homme described in this review were supported by the Juvenile Diabetes Research Foundation International, the Canadian Diabetes Association, the National Cancer Institute of Canada, and in collaboration with Dr AN Theofilopoulos of the Scripps Research Institute, La Jolla, CA by NIH Grants AR31203, AG15061, and AR39555. We thank Vical Inc. (San Diego, CA) for providing the VR1255 expression plasmid used in some of the studies described in the review.
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