{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T18:32:54Z","timestamp":1696357974053},"reference-count":26,"publisher":"The Journal of Rheumatology","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>Previous studies suggested a role for the death decoy receptor 3 (DcR3) in the pathogenesis of adult systemic lupus erythematosus (SLE). We investigated the role of DcR3 in juvenile-onset SLE, to identify polymorphisms that might alter the function of this protein.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>DcR3 was measured in the serum of 61 patients with juvenile SLE. The coding region of the <jats:italic>DcR3<\/jats:italic> gene was sequenced in 100 juvenile and 103 adult patients with SLE, together with 500 healthy controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>DcR3 was elevated in the serum of juvenile patients with active SLE disease (440.8 \u00b1 169.1 pg\/ml), compared to patients with inactive disease (122.6 \u00b1 28.05 pg\/ml; p = 0.0014) and controls (69.27 \u00b1 20.23 pg\/ml; p = 0.0009). DNA sequencing identified 2 novel missense mutations: c.C167T (p.T56I) in an adult SLE patient and c.C364T (p.H122Y) in a juvenile patient. Recombinant proteins containing these mutations exhibited altered binding kinetics to FasL and they significantly increased lymphocyte proliferation, compared to the wild-type protein (p &lt; 0.05). The adult patient with SLE carrying the p.T56I mutation had significantly increased lymphocyte proliferation compared to 3 SLE controls matched for age, sex, and disease severity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>DcR3 may play an etiologic role in SLE through either elevated serum levels of wild-type DcR3 or normal levels of gain-of-function DcR3 proteins that increase lymphocyte proliferation.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.121285","type":"journal-article","created":{"date-parts":[[2013,6,2]],"date-time":"2013-06-02T00:49:20Z","timestamp":1370134160000},"page":"1316-1326","source":"Crossref","is-referenced-by-count":5,"title":["DcR3 Mutations in Patients with Juvenile-onset Systemic Lupus Erythematosus Lead to Enhanced Lymphocyte 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