Fig. 3.

Transcriptome analysis identified myelin sheath pathological signature in mutant FUS iPSC-derived OPCs. Principal component analysis (PCA) of FUS^R521H mutant (a) and FUS^P525L mutant (d) and their respective isogenic controls (N ≥ 3 independent differentiations). Ranked gene set enrichment analysis (GSEA) was performed in FUS^R521H mutant OPCs (b) and FUS^P525L mutant OPCs (e) and their respective isogenic controls using the online tool WebGestalt. The top ten significantly enriched gene ontology (GO) terms of upregulated genes in biological process (BP), cellular component (CC), and molecular function (MF) are presented. Normalized expression from RNAseq data of myelin sheath-related genes in FUS^R521H mutant (c) and FUS^P525L mutant (f) (N ≥ 3 independent differentiations). Statistical analyses were performed by two-way ANOVA with the Bonferroni’s multiple comparisons test (c and f). Data are represented as mean ± SD. *p < 0.05; **p < 0.01 ****p < 0.0001