TDP43 (aka TARDBP) was originally identified as a protein which binds to the transactivation response (TAR) sequence found in the long terminal repeat of the HIV-1 virus genome. TDP43 contains two copies of a ~90 amino acid RNA recognition motif (RRM) found in many proteins which bind single stranded RNA. TDP-43 localizes to the nucleus. In patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis, it is absent from the nucleus of affected neurons but it is the primary component of cytoplasmic ubiquitin-positive inclusion bodies. Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen. Hyperphosphorylated, ubiquitinated, and cleaved in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.