TAR-DNA binding protein 43 (TDP-43) encoding a multi-functional DNA/RNA binding protein, is involved in many events of RNA processing.Recent studies have discovered more than 30 mutations in TDP-43 among patients with TDP-43 proteinopathy, a group of common neurodegenerative diseases, including frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS).FTLD and ALS are devastating diseases that cause impairments in movement, language and cognitive functions.FTLD is among the leading cause of dementia among people younger than 65 years of age.