Extended Data Fig. 4. HSV-1 propagation in immortalized B cells and iPSCs from patients with SNORA31 mutations.

A) Quantification of HSV-1 (strain KOS) in EBV-immortalized B cells from patients (P2, P4, P5), a TRIF^−/− patient and a STAT1^−/− patient, and healthy controls, at the indicated time points post infection with a MOI of 0.1. HSV-1 titers were determined by the TCID₅₀ virus titration method. Means and standard deviations from n=3 independent experiments are shown. B) HSV-1 (strain KOS) abundance, as determined by measurements of GFP-capsid expression in iPSCs (left) at the indicated time points post infection with a MOI of 0.1, for three SNORA31-mutated patients (P2, P3, P5), a TLR3^−/− patient and a STAT1^−/− patient, and hESCs from a healthy control (H9 line). HPSC-derived cortical neurons from a TLR3^−/− patient and a healthy control were assessed in the same assay (right). Mean values from n=2 independent experiments are shown. C, D) Expression levels of the VZV ORF40 (C) and ORF63 (D) transcripts, as measured by RT-qPCR on SV40-fibroblasts from patients (P2-P5), a TLR3^−/− patient and a STAT1^−/− patient, and healthy controls (C1 and C2), 48 h post exposure to VZV-infected MeWo cells (VZV) or MeWo cells that were not infected (N.I.). The data are expressed relative to GAPDH expression. Means and standard deviations from n=3 independent experiments are shown.