Fig. 4

Sanroque mice recapitulate some features of the R688* variant phenotype and develop severe hyperinflammation upon challenge. a Serum concentrations of cytokines TNF, IFNγ, IL-17A, CXCL9, IL-10, IL-6, IL-2 in sanroque mice (n = 4) and control littermates (n = 5). *p < 0.05 (unpaired t-test). b Number of blood neutrophils and lymphocytes in sanroque mice (n = 8) and control littermates (n = 8). ***0.001 < p < 0.0001 (unpaired t-test). c Number of platelets in sanroque mice (n = 11) and control littermates (n = 10) **p < 0.01 (unpaired t-test). d Concentration of serum soluble CD25 (sCD25) in sanroque mice (n = 9) and control littermates (n = 8). ***0.001 < p < 0.0001 (unpaired t-test). e Serum concentration of the liver enzymes aspartate transaminase (AST) and alanine transaminase (ALT) in sanroque mice (n = 6) and littermate controls (n = 12). ****p < 0.001 and **p < 0.01 (unpaired t-test). f Number of splenic immune cell subsets in sanroque chimeras (n = 11) and control chimeras (n = 6). *p < 0.05 and **p < 0.01 (unpaired t-test). g Percentage of splenic regulatory T cells (Treg) and T follicular helper cells (Tfh) in sanroque (n = 11) and control (n = 6) chimeras. *p < 0.05 and **p < 0.01 (unpaired t-test). h Contour plot of CD4⁺ and CD8⁺ T cell differentiation in sanroque and control chimeras. EM: effector memory; CM: central memory; N: naive. i Immunophenotyping of liver derived CD45⁺ cells in sanroque (n = 5) and control chimeric mice (n = 5). *p < 0.05 and **p < 0.01 (unpaired t-test). j Serum concentrations of TNF and IL-10 and k body weight of sanroque and control chimeras treated with 50 μg ODN-1826 CpG or vehicle control every 2 days for 8 days. *p < 0.05, **p < 0.01, ***0.001 < p < 0.0001, ****p < 0.001 (one-way ANOVA with Dunnett’s multiple comparisons test). Data shown are accumulated from three independent experiments (d), two experiments (a–c, e, f, j, k), or representative for two experiments (g–i). When applicable, mean and/or SEM are depicted. Source data are provided as a Source Data file