{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"institution":[{"name":"bioRxiv"}],"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T20:02:24Z","timestamp":1768507344369,"version":"3.49.0"},"posted":{"date-parts":[[2023,7,27]]},"group-title":"Immunology","reference-count":80,"publisher":"openRxiv","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"accepted":{"date-parts":[[2023,7,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                <jats:p>\n                  Patients afflicted with STING gain-of-function mutations frequently present with debilitating interstitial lung disease (\n                  <jats:bold>ILD<\/jats:bold>\n                  ) that is recapitulated in mice expressing the STING\n                  <jats:sup>V154M<\/jats:sup>\n                  mutation (\n                  <jats:bold>VM<\/jats:bold>\n                  ). Prior radiation chimera studies revealed an unexpected and critical role for non-hematopoietic cells in the initiation of ILD. To identify STING-expressing non-hematopoietic cell types relevant to ILD, we generated a conditional knock-in (\n                  <jats:bold>CKI<\/jats:bold>\n                  ) model in which expression of the VM allele was directed to hematopoietic cells, fibroblasts, epithelial cells, or endothelial cells. Only endothelial cell-targeted expression of the mutant allele resulted in the recruitment of immune cells to the lung and the formation of bronchus-associated lymphoid tissue, as seen in the parental VM strain. These findings reveal the importance of endothelial cells as instigators of STING-driven lung disease and suggest that therapeutic targeting of STING inhibitors to endothelial cells could potentially mitigate inflammation in the lungs of SAVI patients or patients afflicted with other ILD-related disorders.\n                <\/jats:p>\n                <jats:sec>\n                  <jats:title>Summary<\/jats:title>\n                  <jats:p>Patients with STING gain-of-function (GOF) mutations develop life-threatening lung autoinflammation. In this study, Gao et al. utilize a mouse model of conditional STING GOF to demonstrate a role for endothelial STING GOF in initiating immune cell recruitment into lung tissues of SAVI mice.<\/jats:p>\n                <\/jats:sec>","DOI":"10.1101\/2023.07.27.550897","type":"posted-content","created":{"date-parts":[[2023,7,28]],"date-time":"2023-07-28T00:25:11Z","timestamp":1690503911000},"source":"Crossref","is-referenced-by-count":3,"title":["Expression of a STING Gain-of-function Mutation in Endothelial Cells Initiates Lymphocytic Infiltration of the Lungs"],"prefix":"10.64898","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5855-1006","authenticated-orcid":false,"given":"Kevin MingJie","family":"Gao","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7322-0811","authenticated-orcid":false,"given":"Kristy","family":"Chiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1158-7164","authenticated-orcid":false,"given":"Filiz T","family":"Korkmaz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9923-9147","authenticated-orcid":false,"given":"Harish Palleti","family":"Janardhan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3544-0474","authenticated-orcid":false,"given":"Chinmay M.","family":"Trivedi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1938-2507","authenticated-orcid":false,"given":"Lee J.","family":"Quinton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastien","family":"Gingras","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3175-609X","authenticated-orcid":false,"given":"Katherine A.","family":"Fitzgerald","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2136-8130","authenticated-orcid":false,"given":"Ann","family":"Marshak-Rothstein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"54368","reference":[{"key":"2023073103000699000_2023.07.27.550897v1.1","doi-asserted-by":"publisher","DOI":"10.1126\/science.aat8657"},{"key":"2023073103000699000_2023.07.27.550897v1.2","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-40055-1"},{"key":"2023073103000699000_2023.07.27.550897v1.3","doi-asserted-by":"crossref","unstructured":"Benmerzoug, S. et al. 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