TARDBP and frontotemporal dementia: These findings, together with our previous reports, suggest that both abnormal cytosolic accumulation as well as nuclear loss of functional TDP-43 collectively contribute to the DNA damage/repair imbalance, as observed in ALS/FTD pathology, which prompted us to develop a conditional cytosolic mislocalization murine Tdp-43 model of ALS by manipulating the murine Tardbp gene to better unravel the pathogenic mechanism of human ALS/FTD.