http://purl.uniprot.org/citations/30772497 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/30772497 | http://www.w3.org/2000/01/rdf-schema#comment | "BackgroundMonogenic interferonopathies are thought to be mediated by type I interferon. For example, a gain-of-function mutation in stimulator of interferon genes (STING; N153S) upregulates type I interferon-stimulated genes and causes perivascular inflammatory lung disease in mice. The equivalent mutation in human subjects also causes lung disease, which is thought to require signaling through the cyclic GMP-AMP synthase (cGAS)-STING pathway and subsequent activation of interferon regulatory factors (IRFs) 3 and 7, type I interferon, and interferon-stimulated genes.ObjectiveWe set out to define the roles of cGAS, IRF3, IRF7, the type I interferon receptor (IFN-α and IFN-β receptor subunit 1 [IFNAR1]), T cells, and B cells in spontaneous lung disease in STING N153S mice.MethodsSTING N153S mice were crossed to animals lacking cGAS, IRF3/IRF7, IFNAR1, adaptive immunity, αβ T cells, and mature B cells. Mice were evaluated for spontaneous lung disease. Additionally, bone marrow chimeric mice were assessed for lung disease severity and survival.ResultsLung disease in STING N153S mice developed independently of cGAS, IRF3/IRF7, and IFNAR1. Bone marrow transplantation revealed that certain features of STING N153S-associated disease are intrinsic to the hematopoietic compartment. Recombination-activating gene 1 (Rag1)-/- STING N153S mice that lack adaptive immunity had no lung disease, and T-cell receptor β chain (Tcrb)-/- STING N153S animals only had mild disease. STING N153S led to a reduction in percentages and numbers of naive and regulatory T cells, as well as an increased frequency of cytokine-producing effector T cells.ConclusionSpontaneous lung disease in STING N153S mice develops independently of type I interferon signaling and cGAS. STING N153S relies primarily on T cells to promote lung disease in mice."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.jaci.2019.01.044"xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Qian W."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Rosen-Wolff A."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Platt D.J."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Miner J.J."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Gerbaulet A."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Luksch H."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Miner C.A."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Bennion B.G."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Stinson W.A."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/author | "Kalugotla G."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/date | "2019"xsd:gYear |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/name | "J Allergy Clin Immunol"xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/pages | "254-266.e8"xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/title | "STING-associated lung disease in mice relies on T cells but not type I interferon."xsd:string |
http://purl.uniprot.org/citations/30772497 | http://purl.uniprot.org/core/volume | "144"xsd:string |
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