NLRP3 and Bruton-type agammaglobulinemia: Following priming by low-doses of LPS or polyI:C, Btk–/– macrophages and PBMCs derived from XLA patients exhibit defective inflammasome activation, caspase-1 activation and IL-1β secretion in response to various activators of NLRP3 such as alum, crystals, ATP and nigericin, but show normal inflammasome activation and IL-1β secretion in response to the AIM2 activator Poly(dA:dT) (Ito et al., 2015; Liu et al., 2017; Weber, 2021).