The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
GSDMD is critical for autoinflammatory pathology in a mouse model of Familial Mediterranean Fever
Open accessAbstract
Pyroptosis is an inflammasome-induced lytic cell death mode, the physiological role of which in chronic inflammatory diseases is unknown. Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease worldwide, affecting an estimated 150,000 patients. The disease is caused by missense mutations in Mefv that activate the Pyrin inflammasome, but the pathophysiologic mechanisms driving autoinflammation in FMF are incompletely understood. Here, we show that Clostridium difficile infection of FMF knock-in macrophages that express a chimeric FMF-associated MefvV726A Pyrin elicited pyroptosis and gasdermin D (GSDMD)–mediated interleukin (IL)-1β secretion. Importantly, in vivo GSDMD deletion abolished spontaneous autoinflammatory disease. GSDMD-deficient FMF knock-in mice were fully protected from the runted growth, anemia, systemic inflammatory cytokine production, neutrophilia, and tissue damage that characterize this autoinflammatory disease model. Overall, this work identifies pyroptosis as a critical mechanism of IL-1β–dependent autoinflammation in FMF and highlights GSDMD inhibition as a potential antiinflammatory strategy in inflammasome-driven diseases.
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Cites
Antiinflammatory Therapy with Canakinumab for Atherosclerotic Disease
Ridker, P. M., Everett, B. M., Thuren, T., MacFadyen, J. G., Chang, W. H., Ballantyne, C., Fonseca, F., Nicolau, J., Koenig, W., Anker, S. D., et al. (2017) · New England Journal of Medicine · doi:10.1056/nejmoa1707914 · Cited by 9,482
Caspase-11 cleaves gasdermin D for non-canonical inflammasome signalling
Kayagaki, N., Stowe, I. B., Lee, B. L., O’Rourke, K., Anderson, K., Warming, S., Cuellar, T., Haley, B., Roose-Girma, M., Phung, Q. T., et al. (2015) · Nature · doi:10.1038/nature15541 · Cited by 3,686
Pore-forming activity and structural autoinhibition of the gasdermin family
Ding, J., Wang, K., Liu, W., She, Y., Sun, Q., Shi, J., Sun, H., Wang, D.-C., & Shao, F. (2016) · Nature · doi:10.1038/nature18590 · Cited by 2,771
The interleukin-1 family: back to the future
Garlanda, C., Dinarello, C. A., & Mantovani, A. (2013) · Immunity · doi:10.1016/j.immuni.2013.11.010 · Cited by 1,945 · cited 2×
“As key regulators of both innate and adaptive immunity, IL-1β and IL-18 not only are central to coordinating antimicrobial host defense, but these cytokines also play central roles in autoimmune and autoinflammatory diseases (Garlanda et al., 2013; Gurung and Kanneganti, 2016; Van Gorp et al., 2017).”
Pharmacological Inhibitors of the NLRP3 Inflammasome
Zahid, A., Li, B., Kombe, A. J. K., Jin, T., & Tao, J. (2019) · Frontiers in Immunology · doi:10.3389/fimmu.2019.02538 · Cited by 671
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Inflammasome-mediated disease animal models reveal roles for innate but not adaptive immunity
Brydges, S. D., Mueller, J. L., McGeough, M. D., Pena, C. A., Misaghi, A., Gandhi, C., Putnam, C. D., Boyle, D. L., Firestein, G. S., Horner, A. A., et al. (2009) · Immunity · doi:10.1016/j.immuni.2009.05.005 · Cited by 339
Cited by
The gasdermins, a protein family executing cell death and inflammation
Broz, P., Pelegrín, P., & Shao, F. (2020) · Nature Reviews Immunology · doi:10.1038/s41577-019-0228-2 · Cited by 1,641
Pyroptosis versus necroptosis: similarities, differences, and crosstalk
Frank, D., & Vince, J. E. (2019) · Cell Death & Differentiation · doi:10.1038/s41418-018-0212-6 · Cited by 1,062
Inflammasomes in neuroinflammatory and neurodegenerative diseases
Voet, S., Srinivasan, S., Lamkanfi, M., & van Loo, G. (2019) · EMBO Molecular Medicine · doi:10.15252/emmm.201810248 · Cited by 761
Self-citationPharmacological Inhibitors of the NLRP3 Inflammasome
Zahid, A., Li, B., Kombe, A. J. K., Jin, T., & Tao, J. (2019) · Frontiers in Immunology · doi:10.3389/fimmu.2019.02538 · Cited by 671
Lee, E., Song, C.-H., Bae, S.-J., Ha, K.-T., & Karki, R. (2023) · Experimental & Molecular Medicine · doi:10.1038/s12276-023-01069-y · Cited by 164
Metadata
- Authors
- Kanneganti, A., Malireddi, R. K. S., Saavedra, P. H. V., Vande Walle, L., Van Gorp, H., Kambara, H., Tillman, H., Vogel, P., Luo, H. R., Xavier, R. J., Chi, H., & Lamkanfi, M.
- Venue
- Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 2018-05-23
- Volume
- 215
- Issue
- 6
- Pages
- 1519-1529
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- OpenAlex
- W2804618431
- Cited by
- 179
- References
- 8
- FWCI
- 7.06
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Kanneganti, A., Malireddi, R. K. S., Saavedra, P. H. V., Vande Walle, L., Van Gorp, H., Kambara, H., Tillman, H., Vogel, P., Luo, H. R., Xavier, R. J., Chi, H., & Lamkanfi, M. (2018). GSDMD is critical for autoinflammatory pathology in a mouse model of Familial Mediterranean Fever. Journal of Experimental Medicine. https://doi.org/10.1084/jem.20172060
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