(A) Comparison of the schematic structure of pyrin proteins of human, mouse, and KI mice in which mouse pyrin is fused with the human B30.2 domains. Growth curves for (B) V726A mutant KI males (WT, n=5; heterozygotes, n=9; and homozygotes, n=4) and (D) M680I mutant KI females (WT, n=9; heterozygotes, n=10; and homozygotes, n=6). Data are as means ± s.d. (C) Hematoxylin and eosin (H&E) stained ear sections (200×) and liver sections (200×) from 8-week-old WT and MefvV726A/V726A mice, and H&E stained section (100×) (bottom) of ankle joints of 4-week-old MefvV726A/V726A mice. (E) H&E stained spleen sections (40×) from 8-week-old WT and MefvV726A/V726A mice. Additional information is provided in Figure S1 and Table S1.