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. 2015 Jul 20;125(8):3103–3116. doi: 10.1172/JCI80924

Figure 6. Sivelestat rescue of SCN iPSC neutrophil maturation is associated with correction of CEBPA, but not CEBPB, expression and NE mislocalization.

Figure 6

(A and B) qPCR assay in sorted promyelocytes/myelocytes. (A) Relative expression of CEBPA. (B) Relative expression of CEBPB. (C) Confocal microscopic localization of primary neutrophil granule proteins NE and MPO in control and SCN iPSCs, and exon 3 mutation–corrected iPSC–derived (SCN15C-derived) promyelocytes. Arrow indicates the colocalization of NE and MPO in primary granules. Scale bars, 10 μm. (D) Quantitation of the correlation of colocalization coefficient between NE and MPO in the primary granules in C. (E) MFI of NE at MPO marked primary granules. In A and B, individual data from 2 or more independent experiments in duplicate are plotted as mean ± SD. In D and E, values of 8 or more cells from 2 independent experiments are presented as mean ± SD. Cont, control.