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Pathogenesis deficiency of the interleukin (IL)-1 receptor antagonist (DIRA) and deficiency of the IL-thirty-three receptor antagonist (DITRA). Blockade of IL-1 signalling by IL-1 receptor antagonist. IL-1 receptor antagonist competes with IL-1 for binding to the IL-1 receptor preventing recruitment of the IL-1 accessory protein and downstream signalling.
