Nucleocytoplasmic transport was defective, leading to an aberrant accumulation of SG-resident proteins within inclusions positive for TDP-43 in the C9orf72-HRE mice model, indicating that abnormal SGs response caused by C9orf72 may lead to TDP-43 proteinopathy in FTD/ALS [81]. This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.