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. 2022 Apr 7;31(21):3652–3671. doi: 10.1093/hmg/ddac080

Figure 5.

Figure 5

The expression and secretion of Cp are increased in heterozygously corrected ATP7B R778L-iPSC-derived hepatocytes. (A) Schematic illustration of the generation of heterozygously corrected ATP7B R778L iPSCs using gene-editing technology. (B) Sequence results around the R778L position of ATP7B gDNA in parental R778L homozygous iPSCs (R778Lhom) and the established gene-edited iPSC clone (R778Lhet). (C) The expression of ATP7B mRNA in WTC11-Hep and ATP7B-edited-Hep on differentiation day 17. Data are shown as the mean ± SEM (n = 3). (D) Representative image of western blot data of ATP7B and GAPDH proteins from parental R778Lhom- and R778Lhet-iPSC-derived hepatocytes on differentiation day 17. (E) Quantified data of ATP7B protein expression after normalization with GAPDH protein. Data are shown as the mean ± SEM (n = 3). (F) Expression of Cp mRNA in R778Lhom-Hep and R778Lhet-Hep detected by RT-qPCR. Data are shown as the mean ± SEM (n = 3). P-values were determined by an unpaired two-tailed Student’s t-test. (G) Representative image of western blot data of Cp and ALB proteins secreted by R778Lhom-Hep and R778Lhet-Hep on differentiation day 17. (H) Quantified data of secreted Cp protein after normalization with ALB protein in R778L hom-Hep and R778L het-Hep. Data are shown as the mean ± SEM (n = 3). P-values were determined by an unpaired two-tailed Student’s t-test.