Matches in SemOpenAlex for { <https://semopenalex.org/work/W4292722416> ?p ?o ?g. }
- W4292722416 abstract "Autoimmune vasculitis is a group of life-threatening diseases, whose underlying pathogenic mechanisms are incompletely understood, hampering development of targeted therapies. Here, we demonstrate that patients suffering from anti-neutrophil cytoplasmic antibodies (ANCA)–associated vasculitis (AAV) showed increased levels of cGAMP and enhanced IFN-I signature. To identify disease mechanisms and potential therapeutic targets, we developed a mouse model for pulmonary AAV that mimics severe disease in patients. Immunogenic DNA accumulated during disease onset, triggering cGAS/STING/IRF3-dependent IFN-I release that promoted endothelial damage, pulmonary hemorrhages, and lung dysfunction. Macrophage subsets played dichotomic roles in disease. While recruited monocyte-derived macrophages were major disease drivers by producing most IFN-β, resident alveolar macrophages contributed to tissue homeostasis by clearing red blood cells and limiting infiltration of IFN-β–producing macrophages. Moreover, pharmacological inhibition of STING, IFNAR-I, or its downstream JAK/STAT signaling reduced disease severity and accelerated recovery. Our study unveils the importance of STING/IFN-I axis in promoting pulmonary AAV progression and identifies cellular and molecular targets to ameliorate disease outcomes." @default.
- W4292722416 created "2022-08-23" @default.
- W4292722416 creator A5001067336 @default.
- W4292722416 creator A5013968213 @default.
- W4292722416 creator A5018454128 @default.
- W4292722416 creator A5018953446 @default.
- W4292722416 creator A5030267616 @default.
- W4292722416 creator A5032791036 @default.
- W4292722416 creator A5041291530 @default.
- W4292722416 creator A5045711240 @default.
- W4292722416 creator A5046366563 @default.
- W4292722416 creator A5047176021 @default.
- W4292722416 creator A5047269468 @default.
- W4292722416 creator A5049305761 @default.
- W4292722416 creator A5053272780 @default.
- W4292722416 creator A5055054841 @default.
- W4292722416 creator A5055207019 @default.
- W4292722416 creator A5063163809 @default.
- W4292722416 creator A5063294321 @default.
- W4292722416 creator A5063701076 @default.
- W4292722416 creator A5066813901 @default.
- W4292722416 creator A5070273513 @default.
- W4292722416 creator A5071291097 @default.
- W4292722416 creator A5071484543 @default.
- W4292722416 creator A5071487336 @default.
- W4292722416 creator A5085018653 @default.
- W4292722416 creator A5085320176 @default.
- W4292722416 creator A5089640692 @default.
- W4292722416 creator A5091449075 @default.
- W4292722416 date "2022-08-23" @default.
- W4292722416 modified "2023-10-16" @default.
- W4292722416 title "Monocyte-derived macrophages aggravate pulmonary vasculitis via cGAS/STING/IFN-mediated nucleic acid sensing" @default.
- W4292722416 cites W137802152 @default.
- W4292722416 cites W1483883929 @default.
- W4292722416 cites W1484645691 @default.
- W4292722416 cites W1559117436 @default.
- W4292722416 cites W1585554327 @default.
- W4292722416 cites W1595642508 @default.
- W4292722416 cites W1821971821 @default.
- W4292722416 cites W1919718846 @default.
- W4292722416 cites W1975923531 @default.
- W4292722416 cites W1979058252 @default.
- W4292722416 cites W2018105494 @default.
- W4292722416 cites W2023134552 @default.
- W4292722416 cites W2035232303 @default.
- W4292722416 cites W2036564362 @default.
- W4292722416 cites W2043756611 @default.
- W4292722416 cites W2055063197 @default.
- W4292722416 cites W2058277625 @default.
- W4292722416 cites W2060004514 @default.
- W4292722416 cites W2066586758 @default.
- W4292722416 cites W2074908076 @default.
- W4292722416 cites W2092288031 @default.
- W4292722416 cites W2097612833 @default.
- W4292722416 cites W2098079184 @default.
- W4292722416 cites W2099610218 @default.
- W4292722416 cites W2100820895 @default.
- W4292722416 cites W2101412709 @default.
- W4292722416 cites W2104299175 @default.
- W4292722416 cites W2105053798 @default.
- W4292722416 cites W2119745575 @default.
- W4292722416 cites W2119864130 @default.
- W4292722416 cites W2121602116 @default.
- W4292722416 cites W2131423819 @default.
- W4292722416 cites W2133402546 @default.
- W4292722416 cites W2133779525 @default.
- W4292722416 cites W2137328170 @default.
- W4292722416 cites W2140431074 @default.
- W4292722416 cites W2141225393 @default.
- W4292722416 cites W2143558168 @default.
- W4292722416 cites W2147350349 @default.
- W4292722416 cites W2152347367 @default.
- W4292722416 cites W2154171253 @default.
- W4292722416 cites W2159470225 @default.
- W4292722416 cites W2162977951 @default.
- W4292722416 cites W2164150624 @default.
- W4292722416 cites W2168197335 @default.
- W4292722416 cites W2170295495 @default.
- W4292722416 cites W2186721808 @default.
- W4292722416 cites W2270059029 @default.
- W4292722416 cites W2317111303 @default.
- W4292722416 cites W2499712993 @default.
- W4292722416 cites W2562980016 @default.
- W4292722416 cites W2597211755 @default.
- W4292722416 cites W2603542690 @default.
- W4292722416 cites W2607382700 @default.
- W4292722416 cites W2626179297 @default.
- W4292722416 cites W2733733381 @default.
- W4292722416 cites W2749547992 @default.
- W4292722416 cites W2758637781 @default.
- W4292722416 cites W2767399558 @default.
- W4292722416 cites W2773262582 @default.
- W4292722416 cites W2796581040 @default.
- W4292722416 cites W2810612816 @default.
- W4292722416 cites W2905503365 @default.
- W4292722416 cites W2913960606 @default.
- W4292722416 cites W2966261467 @default.
- W4292722416 cites W2978415664 @default.
- W4292722416 cites W2998119439 @default.
- W4292722416 cites W3005777482 @default.