The DOI matches its Crossref record; 53 of the 53 references it cites were found in a registry or an index.
A Mutation in the Nlrp3 Gene Causing Inflammasome Hyperactivation Potentiates Th17 Cell-Dominant Immune Responses
Open accessAbstract
Summary Missense mutations of NLRP3 gene (CIAS1) are associated with autoinflammatory disorders characterized with excessive production of IL-1β. Here we analyzed the immune responses of knock-in mice carrying a point mutation of NLRP3 associated with Muckle-Wells Syndrome. We found that antigen presenting cells (APCs) from such mice produce massive amounts of IL-1β and IL-18 upon stimulation with TLR ligands in the absence of ATP. This is likely due to a diminished inflammasome activation threshold that allows a response to the small amount of TLR ligand entering the cell without ATP pulse. Moreover, the NLRP3 knock-in (KI) mice exhibited spontaneous and contactant-induced skin inflammation characterized by neutrophil infiltration and Th17-dominant response, which was originated from hematopoietic cells. The inflammation of KI mice was resulted from excess IL-1β production from APCs which augments Th17 differentitation. These results demonstrate that NLRP3 mutation leads to inflammasome hyper-activation and consequently Th17-dominant infammation in autoinflammatory diseases.
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Hooper, L. V., Littman, D. R., & Macpherson, A. J. (2012) · Science · doi:10.1126/science.1223490 · Cited by 4,173
The NLRP3 Inflammasome: an overview of mechanisms of activation and regulation
Kelley, N., Jeltema, D., Duan, Y., & He, Y. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20133328 · Cited by 3,539
Mechanism and Regulation of NLRP3 Inflammasome Activation
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Metadata
- Authors
- Meng, G., Zhang, F., Fuss, I., Kitani, A., & Strober, W.
- Venue
- Immunity
- Publisher
- Elsevier BV
- Published
- 2009-06
- Volume
- 30
- Issue
- 6
- Pages
- 860-874
- ISSN
- 1074-7613 (ISSN-L)
- OpenAlex
- W1976926965
- Cited by
- 366
- References
- 53
- FWCI
- 12.36
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Meng, G., Zhang, F., Fuss, I., Kitani, A., & Strober, W. (2009). A Mutation in the Nlrp3 Gene Causing Inflammasome Hyperactivation Potentiates Th17 Cell-Dominant Immune Responses. Immunity. https://doi.org/10.1016/j.immuni.2009.04.012
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