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Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease leading to degeneration of motor neurons (MNs). Epigenetic modification of gene expression is increasingly recognized as potential disease mechanism. In the present study we generated motor neurons from induced pluripotent stem cells from ALS patients carrying a mutation in the fused in sarcoma gene (FUS) and analyzed expression and promoter methylation of the FUS gene and expression of DNA methyltransferases ( DNMTs ) compared to healthy control cell lines. While mutant  FUS  neural progenitor cells (NPCs) did not show a difference in  FUS  and  DNMT  expression compared to healthy controls, differentiated mutant  FUS  motor neurons showed significantly lower  FUS  expression, higher  DNMT  expression and higher methylation of the proximal  FUS  gene promoter. Immunofluorescence revealed perceived proximity of cytoplasmic  FUS  aggregates in ALS MNs together with 5-methylcytosin (5-mC). Targeting disturbed methylation in ALS may therefore restore transcriptional alterations and represent a novel therapeutic strategy.

Methylation and Expression of Mutant FUS in Motor Neurons Differentiated From Induced Pluripotent Stem Cells From ALS Patients