Gene Interaction at HLA-DQ Enhances Autoantibody Production in Primary Sjögren's Syndrome

• Published in: Science (American Association for the Advancement of Science) Vol. 232; no. 4754; pp. 1145 - 1147
• Main Authors: Harley, John B., Reichlin, Morris, Arnett, Frank C., Alexander, Elaine L., Bias, Wilma B., Provost, Thomas T.
• Format: Journal Article
• Language: English
• Published: Washington, DC The American Association for the Advancement of Science 30.05.1986; American Association for the Advancement of Science
• Subjects: Alleles; Animals; Antibodies; Antigens; Autoantibodies; Autoantibodies - biosynthesis; Autoantibodies - genetics; Autoimmune diseases; Biological and medical sciences; Dry eye syndromes; Gene interaction; Genetics; Heterozygotes; Histochemistry; Histocompatibility antigens; Histocompatibility Antigens Class II - genetics; Histocompatibility Antigens Class II - immunology; HLA antigens; HLA histocompatibility antigens; HLA-DQ Antigens; HLA-DR Antigens; Immunologic research; Immunological research; Medical disorders; Medical sciences; Mice; Molecules; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Sjogren's syndrome; Sjogren's Syndrome - genetics; Sjogren's Syndrome - immunology; Systemic lupus erythematosus; Human; Immunopathology; HLA-System; Eye disease; Skin disease; Gene interaction; Autoantibody; ENT disease; Biosynthesis; Sjogren syndrome
• ISSN: 0036-8075; 1095-9203
• DOI: 10.1126/science.3458307

Primary Sjögren's syndrome is an autoimmune disorder characterized by dryness of the mouth and eyes. The human leukocyte antigen (HLA) locus DQ is related to the primary Sjögren's syndrome autoantibodies that bind the RNA proteins Ro/SSA and La/SSB. Both DQ1 and DQ2 alleles are associated with high concentrations of these autoantibodies. An analysis of all possible combinations at DQ has shown that the entire effect was due to heterozygotes expressing the DQ1 and DQ2 alleles. These data suggest that gene interaction between DQ1 and DQ2 (or alleles at associated loci), possibly from gene complementation of trans-associated surface molecules, influences the autoimmune response in primary Sjögren's syndrome.