@article {463,
	title = {Pro-inflammation Associated with a Gain-of-Function Mutation (R284S) in the Innate Immune Sensor STING.},
	journal = {Cell Rep},
	volume = {23},
	year = {2018},
	month = {2018 Apr 24},
	pages = {1112-1123},
	abstract = {<p>The cellular sensor stimulator of interferon genes (STING) initiates type I interferon (IFN) and cytokine production following association with cyclic dinucleotides (CDNs) generated from intracellular bacteria or via a cellular synthase, cGAS, after binding microbial or self-DNA. Although essential for protecting the host against infection, unscheduled STING signaling is now known to be responsible for a variety of autoinflammatory disorders. Here, we report a gain-of-function mutation in STING (R284S), isolated from a patient who did not require CDNs to augment activity and who manifested a constitutively active phenotype. Control of the Unc-51-like autophagy activating kinase 1 (ULK1) pathway, which has previously been shown to influence STING function, was potently able to suppress STING (R284S) activity to alleviate~cytokine production. Our findings add to the growing~list of inflammatory syndromes associated with spontaneous STING signaling and provide a therapeutic strategy for the treatment of STING-induced inflammatory disease.</p>},
	issn = {2211-1247},
	doi = {10.1016/j.celrep.2018.03.115},
	author = {Konno, Hiroyasu and Chinn, Ivan K and Hong, Diana and Orange, Jordan S and Lupski, James R and Mendoza, Alejandra and Pedroza, Luis A and Barber, Glen N}
}
