Microarray analysis of EDI MoDCs stimulated with CD40L and CD40L plus IFN-γ. MoDCs from healthy (N1-N3) and EDI (EDI patient 1; EDI1-1 and EDI1-2 from 2 independent cultures: EDI patient 2; EDI2) subjects were stimulated with CD40L alone or CD40L plus IFN-γ for 24 hours. Each row represents the ratio of expression in stimulated versus unstimulated cells for each gene, and each column represents the data from an independent stimulation experiment. The color bar shows the magnitude of gene expression changes on a log scale: significantly induced (red), unchanged (black), or repressed (green). (A) Genes that show consistent regulation in normal MoDCs in response to CD40L but abnormal regulation in EDI MoDCs. A hierarchic cluster map of 400 probe sets that show consistent regulation in normal MoDCs (> 2-fold; P < .01) but significantly different regulation in EDI MoDCs (ANOVA at P < .01). (B) Addition of IFN-γ restores a portion of the CD40L expression signature in EDI MoDCs. A hierarchic cluster map of the expression patterns for the 400 probe sets from panel A in response to CD40L plus IFN-γ. Note that many of the probe sets show similar expression in EDI and normal samples, illustrating the effects of IFN-γ on complementing the CD40L expression signature in EDI MoDCs. (C) EDI MoDCs fail to up-regulate a subset of CD40L-induced genes even in the presence of IFN-γ. A number of probe sets (53) are shown that passed the following filter criteria: significantly regulated in all 3 normal CD40L plus IFN-γ cultures (P < .01); and no significant regulation in any of the 3 EDI CD40L plus IFN-γ cultures. Note that for this analysis any ratio values with P > .01 were set to log (ratio) = 0.