Based on the above findings, we explored the relationship between the genotype and phenotype of ALS disease and discovered that nucleocytoplasmic transport defects were detected in hiPSCs-MNs of C9orf72 and TDP43 mutant patients, but not in SOD1 ones, indicating that different genotypes may cause MNs degeneration through different mechanisms. Here, TARDBP is linked to amyotrophic lateral sclerosis.