ArticleProceedings of the National Academy of Sciences of the United States of America2022
Radioresistant cells initiate lymphocyte-dependent lung inflammation and IFNγ-dependent mortality in STING gain-of-function mice.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 38 citations in OpenAlex.
- Endothelial cell expression of STINGThe Journal of investigative dermatology · 2026Article
- Interstitial lung disease and the STING pathway.The Journal of clinical investigation · 2026Review
- Autoinflammatory syndromes of STING and TREX1 dysfunction.The Journal of clinical investigation · 2026Review
- STING-STAT3-SOX18 Axis Drives EndMT and Epigenetic Reprogramming in SAVI Lung Fibrosis.bioRxiv : the preprint server for biology · 2026Article
- First successful allogeneic hematopoietic stem cell transplantation in STING-associated vasculopathy of infancy-A case report.Molecular therapy. Advances · 2026Article
- SAVI: molecular mechanisms, clinical spectrum and precision medicine approaches beyond type-I IFN.Frontiers in immunology · 2026Review
- Lymphopenia drives T cell exhaustion in immunodeficient STING gain-of-function mice.EMBO molecular medicine · 2025Article
- Article
- PARP7 inhibits type I interferon signaling to prevent autoimmunity and lung disease.The Journal of experimental medicine · 2025Article
- Article
- Tissue inflammation induced by constitutively active STING is mediated by enhanced TNF signaling.eLife · 2025Article
- Dynamic STING repression orchestrates immune cell development and function.Science immunology · 2025Article
- DNA-sensing pathways in health, autoinflammatory and autoimmune diseases.Nature immunology · 2024Review
- The commoneLife · 2024Article
- A novel STING variant triggers endothelial toxicity and SAVI disease.The Journal of experimental medicine · 2024Article
- Activation of autoreactive lymphocytes in the lung by radioresistant cells expressing a STING gain-of-function mutation.JCI insight · 2024Article
- The commonbioRxiv : the preprint server for biology · 2024Article
- Endothelial cell expression of a STING gain-of-function mutation initiates pulmonary lymphocytic infiltration.Cell reports · 2024Article
- Single-cell RNA-sequencing of PBMCs from SAVI patients reveals disease-associated monocytes with elevated integrated stress response.Cell reports. Medicine · 2023Article
- Regulation of cGAS and STING signaling during inflammation and infection.The Journal of biological chemistry · 2023Review
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
Pediatric patients with constitutively active mutations in the cytosolic double-stranded-DNA-sensing adaptor STING develop an autoinflammatory syndrome known as STING-associated vasculopathy with onset in infancy (SAVI). SAVI patients have elevated interferon-stimulated gene expression and suffer from interstitial lung disease (ILD) with lymphocyte predominate bronchus-associated lymphoid tissue (BALT). Mice harboring SAVI mutations (STING V154M [VM]) that recapitulate human disease also develop lymphocyte-rich BALT. Ablation of either T or B lymphocytes prolongs the survival of SAVI mice, but lung immune aggregates persist, indicating that T cells and B cells can independently be recruited as BALT. VM T cells produced IFNγ, and IFNγR deficiency prolonged the survival of SAVI mice; however, T-cell-dependent recruitment of infiltrating myeloid cells to the lung was IFNγ independent. Lethally irradiated VM recipients fully reconstituted with wild type bone-marrow-derived cells still developed ILD, pointing to a critical role for VM-expressing radioresistant parenchymal and/or stromal cells in the recruitment and activation of pathogenic lymphocytes. We identified lung endothelial cells as radioresistant cells that express STING. Transcriptional analysis of VM endothelial cells revealed up-regulation of chemokines, proinflammatory cytokines, and genes associated with antigen presentation. Together, our data show that VM-expressing radioresistant cells play a key role in the initiation of lung disease in VM mice and provide insights for the treatment of SAVI patients, with implications for ILD associated with other connective tissue disorders.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.