TNFR2 induced priming of the inflammasome leads to a RIPK1-dependent cell death in the absence of XIAP

Knop, Janin; Spilgies, Lisanne M; Rufli, Stefanie; Reinhart, Ramona; Vasilikos, Lazaros; Yabal, Monica; Owsley, Erika; Jost, Philipp J; Marsh, Rebecca A; Wajant, Harald; Robinson, Mark D; Kaufmann, Thomas; Wong, W Wei-Lynn

doi:10.1038/s41419-019-1938-x

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Knop, J., Spilgies, L. M., Rufli, S., Reinhart, R., Vasilikos, L., Yabal, M., Owsley, E., Jost, P. J., Marsh, R. A., Wajant, H., Robinson, M. D., Kaufmann, T., & Wong, W. W.-L. (2019). TNFR2 induced priming of the inflammasome leads to a RIPK1-dependent cell death in the absence of XIAP. Cell Death and Disease, 10(10), 700. https://doi.org/10.1038/s41419-019-1938-x

Abstract

The pediatric immune deficiency X-linked proliferative disease-2 (XLP-2) is a unique disease, with patients presenting with either hemophagocytic lymphohistiocytosis (HLH) or intestinal bowel disease (IBD). Interestingly, XLP-2 patients display high levels of IL-18 in the serum even while in stable condition, presumably through spontaneous inflammasome activation. Recent data suggests that LPS stimulation can trigger inflammasome activation through a TNFR2/TNF/TNFR1 mediated loop in xiap$^{-/-}$