Finally, pathological cytoplasmic FUS in the context of FTLD – but not ALS – is reportedly associated with reduced methylation of FUS at the RGG3 domain (next to the PY-NLS) [177], and this hypomethylation influences the interaction between FUS and transportin 1 – which could in turn contribute to abnormal cytoplasmic FUS accumulation [177]. This evidence concerns the gene TNPO1 and amyotrophic lateral sclerosis.