TDP-43/TARDBP Proteins and Enzymes

TARDBP, also known as TAR DNA binding protein, is a DNA and RNA-binding protein which regulates transcription and splicing. TARDBP is also involved in the regulation of CFTR splicing and promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. TARD-BP may also be involved in microRNA biogenesis, apoptosis and cell division. TARDBP can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat.