Figure 2. Expansion of CD11b⁺ cells and gain of function B30.2 mutations with a gene dosage effect.

(A) Flow cytometry analyses of spleens from 4- and 12-week-old Mefv^V726A/V726A mice. Total splenocytes were analyzed for the relative frequencies of lymphoid (CD3 and CD19 for T cells and B cells, respectively, first row) and myeloid cells (CD11b, second row). Within CD11b⁺-gated myeloid cells, Ly-6G⁺ neutrophils and F4/80⁺ monocytes or macrophages were analyzed (third row). Numbers indicate percentage of total cells in respective quadrants or gates. Data are representative of three independent experiments. (B) Generation of hemizygous (Mefv^V726A/−) KI mice for the mutant B30.2 domain. Peripheral blood cells were analyzed for CD11b⁺ cells from their 8-week-old offspring. Data are representative of three independent experiments. (C) Body weights of six 8-week-old males of Mefv^+/+, Mefv^V726A/+, Mefv^V726A/V726A, Mefv^V726A/−, and Mefv^−/−. (D) Expression of pyrin in peritoneal macrophages from M680I-KI and pyrin-deficient mice following 24 h of culture. Additional information is provided in Figure S2.