ArticleNature immunology2021
Human autoinflammatory disease reveals ELF4 as a transcriptional regulator of inflammation.
Article in Nature immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.
What it found
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Who cites it
50 citing papers in PubMed, 98 citations in OpenAlex.
- Regulation of Inducible Nitric Oxide Synthase (NOS2) Expression in Healthy and Inflamed Bowel: A Narrative Review.International journal of molecular sciences · 2026Review
- Metabolically engineered probiotic OMVs as nanovaccine mediating sequential immunomodulation for chronic bone infection immunotherapy.Cell reports. Medicine · 2026Article
- Behçet's spectrum disorders: genetic and immunological insights into an emerging disease concept.World journal of pediatrics : WJP · 2026Review
- A novel ELF4 gene variant disrupts T and NK cell function in a patient with immune thrombocytopenia (ITP).Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2026Article
- Bioinformatic validation of LCN2 as a common hub gene for ferroptosis, pyroptosis and necroptosis in ulcerative colitis.BMC gastroenterology · 2026Article
- Transcriptomic comparison of human intestinal organoids and Caco-2 cells in modeling nutrient absorption: insights from infant formula and breast milk.NPJ science of food · 2026Article
- Article
- A truncatingFrontiers in immunology · 2026Article
- Roles of endothelial cells during infection.Current opinion in immunology · 2025Review
- Epithelial pyroptosis-induced TREM1Cell death discovery · 2025Article
- Review
- Novel pathogenic variant in the deficiency in ELF4, X-linked (DEX): case report and literature review.Orphanet journal of rare diseases · 2025Review
- 12/15-lipoxygenase orchestrates murine wound healing via PPARγ-activating oxylipins acting holistically to dampen inflammation.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Two novel frameshift variations in theTranslational pediatrics · 2025Article
- Multi-omics and functional characterization of the tumor-killing capacity of Imiquimod-activated plasmacytoid dendritic cells.iScience · 2025Article
- ELF4 improves sepsis-induced myocardial injury by regulating STING signaling-mediated T cells differentiation.Cell biology and toxicology · 2025Article
- A zinc transporter drives glioblastoma progression via extracellular vesicles-reprogrammed microglial plasticity.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Transcription factor ELF4 in physiology and diseases: Molecular roles and clinical implications.Genes & diseases · 2025Review
- Progressive accumulation of circulating CD27Experimental & molecular medicine · 2025Article
- Gut-derived macrophages link intestinal damage to brain injury after cardiac arrest through TREM1 signaling.Cellular & molecular immunology · 2025Article
Corrections and comments
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Authors and funding
18 authors at 3 institutions in 3 countries.
Funding
Abstract
Transcription factors specialized to limit the destructive potential of inflammatory immune cells remain ill-defined. We discovered loss-of-function variants in the X-linked ETS transcription factor gene ELF4 in multiple unrelated male patients with early onset mucosal autoinflammation and inflammatory bowel disease (IBD) characteristics, including fevers and ulcers that responded to interleukin-1 (IL-1), tumor necrosis factor or IL-12p40 blockade. Using cells from patients and newly generated mouse models, we uncovered ELF4-mutant macrophages having hyperinflammatory responses to a range of innate stimuli. In mouse macrophages, Elf4 both sustained the expression of anti-inflammatory genes, such as Il1rn, and limited the upregulation of inflammation amplifiers, including S100A8, Lcn2, Trem1 and neutrophil chemoattractants. Blockade of Trem1 reversed inflammation and intestine pathology after in vivo lipopolysaccharide challenge in mice carrying patient-derived variants in Elf4. Thus, ELF4 restrains inflammation and protects against mucosal disease, a discovery with broad translational relevance for human inflammatory disorders such as IBD.
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Registered trials
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.