Description
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