Fig. 8. Rescue of antiviral immunity in iPSCs-derived neurons.
A Induction of IFNβ mRNA after 1 h of Poly(I:C) stimulation in neurons from WWP2 p.R841H (red, N = 2), WWP2 p.H841R (grey, N = 2) individuals and WWP2 p.R841 controls (black, N = 3). B Induction of IFNβ mRNA after 1 h of Poly(I:C) stimulation in neurons from WWP2 p.R841 (black, N = 2), WWP2 p.R841H (grey, N = 2) individuals and WWP2 p.H841 controls (red, N = 4). Results represent the mean ± standard error of 1 experiment among 2. C Quantification of the number of viral genome in neurons from WWP2 p.R841H (red, N = 2), WWP2 p.H841R (grey, N = 2) individuals, and WWP2 p.R841 controls (black, N = 4), without or with an IFNα pretreatment (+IFN). D Quantification of the number of viral genome in neurons from WWP2 p.R841 (black, N = 2), WWP2 p.R841H (grey, N = 1) individuals and WWP2 p.H841 controls (black, N = 7) without or with an IFNα pretreatment (+IFN). E Quantification of the number of plaque-forming units (pfu) by plaque-titration after infection of neurons from WWP2 p.R841H (red, N = 2), WWP2 p.H841R (grey, N = 2) individuals and WWP2 p.R841 controls (black, N = 3) without or with an IFNα2b pretreatment (+IFN). F Quantification of the number of plaque-forming units (pfu) by plaque-titration after infection of neurons from WWP2 p.R841 (black, N = 2), WWP2 p.R841H (grey, N = 2) individuals and WWP2 p.H841 controls (black, N = 4) without or with an IFNα2b pretreatment (+IFN). G Quantification of the number of plaque-forming units (pfu) by plaque-titration at different time after infection of neurons from WWP2 p.R841H (red, N = 2), WWP2 p.H841R (grey, N = 2) individuals and WWP2 p.R841 controls (black, N = 3). A significant effect (*) of the rescue (grey) compared to allele carrier (red) (p = 2.41e-5) was found using a linear regression on log10 + 1 transformed pfu with a time-dependent effect. H Quantification of the number of plaque-forming units (pfu) by plaque-titration at different time after infection of neurons from WWP2 p.R841 (black, N = 2), WWP2 p.R841H (grey, N = 2) individuals and WWP2 p.H841 controls (black, N = 4). A significant effect (*) of the mutant induced (pink) compared to control (black) (p = 2.06e-3) was found using a linear regression on log10 + 1 transformed pfu with a time-dependent effect.