FIGURE 2.
Cells from patients with NLRP7 mutations and rare variants display normal to higher pro-IL-1β synthesis and normal processing. Freshly isolated PBMCs (1.5 × 106 cells) were stimulated with LPS for 24 h. Immunoblots of whole cell lysates show the expression of intracellular pro, mature IL-1β relative to β-actin in patients with NLRP7 mutations (A) and rare variants (B). C, recapitulation of the ratios of pro, mature, and secreted IL-1β by the cells of patients divided by control cells (patient pro-IL-1β/control pro-IL-1β) after signal quantification using ImageJ software. When sufficient amount of cells from patients were available, the averages and S.D. were calculated on two to three different Western blots on cellular lysates from the same LPS stimulation.