TDP43 Antibody, pAb, Rabbit

TDP43 (known as TARDBP, TAR DNA-binding protein 43) is a DNA and RNA-binding protein which regulates transcription and splicing. The deduced 414-amino acid TDP43 contains a ribonucleoprotein (RNP)-binding domain and a glycine-rich region. TDP43 protein is located at cell nuclear in normal condition. TDP43 promotes skipping of exon 9 of the CFTR gene and also inhibits human CFTR exon 9 splicing. A hyperphosphorylated, ubiquitinated, and cleaved form of TDP43, known as pathologic TDP43, is the major disease protein in ubiquitin-positive, tau-, and alpha-synuclein-negative frontotemporal dementia (FTLD-U) and amyotrophic lateral sclerosis (ALS). Under pathologic conditions in FTLD-U, TDP43 is likely to be eliminated from nuclei of ubiquitinated inclusion-bearing neurons, a consequence of which may be a loss of TDP43 nuclear functions. TDP43 has been implicated in AIDS disease.GenScript Rabbit Anti-TDP43 Polyclonal Antibody is developed in rabbit using a KLH-coupled synthetic peptide from within residues 250-300 of human TDP43 protein (Swiss Prot: Q13148).