TDP-43, the transactive response (TAR)-DNA binding Protein with a molecular weight of 43 KDa is encoded by TARDBP gene that located at the chromosome 1. It is a RNA/DNA binding protein that structurally resembles a typical hnRNP protein family member. involved in transcriptional regulation and RNA processing. It is linked to sporadic and familial amyotrophic lateral sclerosis and frontotemporal lobar degeneration. TDP-43 is predominantly nuclear, but it translocates to the cytoplasm under pathological conditions. Cytoplasmic accumulation, phosphorylation, ubiquitination and truncation of TDP-43 are the main hallmarks of TDP-43 proteinopathies.