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. Author manuscript; available in PMC: 2014 Sep 16.
Published in final edited form as: Nat Rev Immunol. 2012 Jul 25;12(8):570–580. doi: 10.1038/nri3261

Table 2. Pathogenic mechanisms in autoinflammatory diseases.

The abnormal folding and accumulation of proteins regulating inflammation can lead to different degrees of cellular stress. The effect of mutations in pyrin and NLRP3 is primarily local, HLA-B27 and XBP1 deficiency can activate the unfolded protein response (UPR) associated with toxicity and enhanced inflammatory responses.

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