ArticleThe Journal of experimental medicine2021
Neutrophil-specific gain-of-function mutations in Nlrp3 promote development of cryopyrin-associated periodic syndrome.
Article in The Journal of experimental medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 46 citations in OpenAlex.
- Targeting CD177: A Novel Therapeutic Strategy for NLRP3-Associated Autoinflammatory Diseases.International journal of molecular sciences · 2026Article
- The NLRP3 Inflammasome: Mechanisms of Activation, Regulation, and Therapeutic Opportunities.MedComm · 2026Review
- S1PR1 signaling biases neutrophils toward long-lived low-inflammatory functional states.bioRxiv : the preprint server for biology · 2026Article
- The expanding role of the NLRP3 inflammasome from periodic fevers to therapeutic targets.Nature immunology · 2025Review
- NLRP3 activation induces BBB disruption and neutrophil infiltration via CXCR2 signaling in the mouse brain.Journal of neuroinflammation · 2025Article
- Decoding Immunobiology Through Genetic Errors of Immunity.Annual review of immunology · 2025Review
- Article
- The discovery of NLRP3 and its function in cryopyrin-associated periodic syndromes and innate immunity.Immunological reviews · 2024Review
- To Gain Insights into the Pathophysiological Mechanisms of the Thrombo-Inflammatory Process in the Atherosclerotic Plaque.International journal of molecular sciences · 2023Review
- Regulated cell death in neutrophils: From apoptosis to NETosis and pyroptosis.Seminars in immunology · 2023Review
- NLRP3 selectively drives IL-1β secretion by Pseudomonas aeruginosa infected neutrophils and regulates corneal disease severity.Nature communications · 2023Article
- The NLRP3 inflammasome: contributions to inflammation-related diseases.Cellular & molecular biology letters · 2023Review
- Neutrophils in Health and Disease: From Receptor Sensing to Inflammasome Activation.International journal of molecular sciences · 2023Review
- Gasdermin D inhibition ameliorates neutrophil mediated brain damage in acute ischemic stroke.Cell death discovery · 2023Article
- The role of NOD-like receptors in innate immunity.Frontiers in immunology · 2023Review
- Uncoupled pyroptosis and IL-1β secretion downstream of inflammasome signaling.Frontiers in immunology · 2023Review
- NLRP3 inflammasome activation in neutrophils directs early inflammatory response in murine peritonitis.Scientific reports · 2022Article
- Review
- Tumor-intrinsic NLRP3-HSP70-TLR4 axis drives premetastatic niche development and hyperprogression during anti-PD-1 immunotherapy.Science translational medicine · 2022Article
- NLRP3 activation in neutrophils induces lethal autoinflammation, liver inflammation, and fibrosis.EMBO reports · 2022Article
Corrections and comments
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Authors and funding
17 authors at 8 institutions in 5 countries.
Funding
Abstract
Gain-of-function mutations in NLRP3 are responsible for a spectrum of autoinflammatory diseases collectively referred to as "cryopyrin-associated periodic syndromes" (CAPS). Treatment of CAPS patients with IL-1-targeted therapies is effective, confirming a central pathogenic role for IL-1β. However, the specific myeloid cell population(s) exhibiting inflammasome activity and sustained IL-1β production in CAPS remains elusive. Previous reports suggested an important role for mast cells (MCs) in this process. Here, we report that, in mice, gain-of-function mutations in Nlrp3 restricted to neutrophils, and to a lesser extent macrophages/dendritic cells, but not MCs, are sufficient to trigger severe CAPS. Furthermore, in patients with clinically established CAPS, we show that skin-infiltrating neutrophils represent a substantial biological source of IL-1β. Together, our data indicate that neutrophils, rather than MCs, can represent the main cellular drivers of CAPS pathology.
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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.