Article
STING-associated vasculopathy develops independently of IRF3 in mice.
The Journal of experimental medicine - 6 Nov 2017
Warner James D, Irizarry-Caro Ricardo A, Bennion Brock G, Ai Teresa L, Smith Amber M, Miner Cathrine A, Sakai Tomomi, Gonugunta Vijay K, Wu Jianjun, Platt Derek J, Yan Nan, Miner Jonathan J
Abstract excerpt
Patients with stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) develop systemic inflammation characterized by vasculopathy, interstitial lung disease, ulcerative skin lesions, and premature death. Autosomal dominant mutations in STING are thought to trigger activation of IRF3 and subsequent up-regulation of interferon (IFN)-stimulated genes (ISGs) in patients with SAVI....
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