Fig. 4

Riluzole treatment stimulates incorporation of the leukodystrophy-associated POLR3B R103H variant in Pol III.

HEK293 cells transfected with FLAG-tagged POLR3B WT or R103H were treated with 12.5 µM of riluzole or vehicle (DMSO) for 20 h, in triplicate. Affinity purification was performed as in Fig. 2. Shown are the 17 POLR3 subunit mean LFQ intensities. Significant fold-decrease values (↓) against the DMSO treated WT and fold-increase values (↑) against the DMSO treated R103H values are indicated in each histogram. Two-way ANOVA statistical analysis was performed by using the log₂-transformed imputated intensities. **: p < 0.01, ****: p < 0.0001; significant main effect of R103H mutation. +: p < 0.05; significant main effect of riluzole. #: p < 0.05, ##: p < 0.01, ###: p < 0.001; Significant effect of riluzole in R103H expressing cell. @: p < 0.05; significant effect of riluzole in WT expressing cell. &&: p < 0.01, &&&: p < 0.001, &&&&: p < 0.0001; significantly different that WT-DMSO.