Figure 1.
SCN-iPSC-derived CD34+CD45+cells show elevated ROS levels and increased nuclear translocation of NRF2. (A) Representative FACS histograms and quantifications of mean fluorescent intensities (MFI) showing increased levels of CellROX Deep Red in SCN HPCs. Data are from 3 independent experiments on 2 clones per genotype. (B) Quantification of the nuclear translocation of NRF2 by immunofluorescence stainings of HPCs showing increased nuclear NRF2 levels in SCN HPCs. The red line indicates the median NRF2 nuclear intensity for the control HPCs. Data are pooled from 2 independent clones and 5 independent experiments. Total cells analyzed: control, n = 765; ELANE-R103L, n = 721; ELANE-I60F, n = 677; HAX1-W44X, n = 574. (C) Expression of the antioxidant glutathione reductase (GSR) in TPM obtained from 2 different iPSC clones and 2 independent experiments. *P < .05, **P < .01, ***P < .001.