TARDBP and amyotrophic lateral sclerosis: Our analysis of ALS patient-derived LCLs as a valid cellular model to study the disease that carries typical features of degenerating MNs in ALS (i.e. protein aggregation, mitochondrial disfunction etc.)[46], also revealed an increased level of nuclear RNA-DNA hybrids in LCL-TDP382 as well as co-localization of S9.6 antibody with a fraction of TDP-43 in the perinuclear area.