ARTHRITIS & RHEUMATOLOGY Vol. 66, No. 1, January 2014, pp 163–172 DOI 10.1002/art.38187 © 2014, American College of Rheumatology Estrogen Receptor Regulates Tripartite Motif–Containing Protein 21 Expression, Contributing to Dysregulated Cytokine Production in Systemic Lupus Erythematosus Siobha ´n Smith, 1 Joan Nı ´ Gabhann, 1 Eoghan McCarthy, 1 Barbara Coffey, 1 Rebecca Mahony, 1 Jennifer C. Byrne, 1 Kevin Stacey, 1 Elizabeth Ball, 2 Aubrey Bell, 2 Gaye Cunnane, 3 Michele F. Doran, 3 Eamonn S. Molloy, 4 Ruth Z. Lee, 5 Brian Harvey, 6 Grainne Kearns, 5 and Caroline A. Jefferies 1 Objective. To examine the role of 17-estradiol in the regulation of the autoantigen tripartite motif– containing protein 21 (TRIM-21) in patients with sys- temic lupus erythematosus (SLE). Methods. Monocytes isolated from healthy con- trol subjects and patients with SLE were stimulated with 17-estradiol and/or the estrogen receptor (ER) antagonist methyl-piperidino-pyrazole dihydro- chloride. TRIM-21, ER, and CREMexpression was determined by real-time polymerase chain reaction (PCR) analysis. MatInspector software was used to identify putative binding sites within the TRIM-21 promoter. ERbinding to the TRIM-21 gene promoter region in monocytes was analyzed by chromatin immu- noprecipitation (ChIP) assay. TRIM-21 and interferon regulatory factor 3 protein levels were analyzed by Western blotting. Results. Real-time PCR analysis demonstrated a role of estrogen in the regulation of TRIM-21 expression in monocytes, which correlated positively with ERgene expression in patients with SLE. Investigations into the human TRIM-21 promoter revealed the presence of an estrogen response element, with ChIP assays confirming ERbinding to this site. Studies into estrogen-induced TRIM-21 expression revealed a hyperresponsiveness of SLE patients to 17-estradiol, which led to the en- hanced levels of TRIM-21 observed in these individuals. Conclusion. Our results demonstrate a role of estrogen in the regulation of TRIM-21 expression through an ER-dependent mechanism, a pathway that we observed to be overactive in SLE patients. Treatment of monocytes with an ERantagonist abrogated estrogen-induced TRIM-21 expression and, as a conse- quence, decreased the expression of interleukin-23. These findings identify TRIM-21 as a novel ER- regulated gene and provide novel insights into the link between estrogen and the molecular pathogenesis of SLE. Systemic lupus erythematosus (SLE) is character- ized by a wide variety of immunologic abnormalities, including abnormal T and B lymphocyte signaling (1), the production of autoantibodies to self antigens (2), and the defective clearance of immune complexes by macrophages (3). In addition, a strong genetic predispo- sition is associated with this condition, with a 9:1 ratio of affected females to affected men (4). This sex imbalance has suggested a role of sex hormones, particularly estro- gen, as important players in the development of SLE. Supported by the Science Foundation Ireland (grant 08/IN.1/ B2091). 1 Siobha ´n Smith, MSc, Joan Nı ´ Gabhann, PhD, Eoghan Mc- Carthy, MB, Barbara Coffey, BSc, Rebecca Mahony, BSc, Jennifer C. Byrne, PhD, Kevin Stacey, PhD, Caroline A. Jefferies, PhD: Royal College of Surgeons in Ireland, Dublin, Ireland; 2 Elizabeth Ball, MB, Aubrey Bell, MD: Belfast Health and Social Care Trust and Belfast City Hospital, Belfast, UK; 3 Gaye Cunnane, PhD, Michele F. Doran, MD: St. James Hospital, Dublin, Ireland; 4 Eamonn S. Molloy, MD: St. Vincent’s University Hospital, Dublin, Ireland; 5 Ruth Z. Lee, MD, Grainne Kearns, MD: Beaumont Hospital, Dublin, Ireland; 6 Brian Harvey, PhD: Royal College of Surgeons in Ireland and Beaumont Hospital, Dublin, Ireland. Address correspondence to Caroline A. Jefferies, PhD, Mo- lecular and Cellular Therapeutics, Royal College of Surgeons in Ireland, 123 St. Stephen’s Green, Dublin 2, Ireland. E-mail: cjefferies @rcsi.ie. Submitted for publication April 22, 2013; accepted in revised form August 29, 2013. 163