Anti-TDP43 TARDBP Antibody

TDP43 was initially identified as a novel cellular protein that bound to HIV-1 virus TAR DNA sequence motifs and acts a transcriptional repressor to the HIV-1 LTR. Later experiments revealed that TDP43 also regulates the splicing of exon 9 of the cystic fibrosis transmembrane conductance regular (CFTR), most likely through the association with the UG repeats at the 3'splice site. 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 (FLTD-U) . TDP43 is not related to TRBP1, and RNA binding protein that binds HIV-1 TAR RNA sequences.

TDP43 antibody was raised against a 15 amino acid synthetic peptide from near the amino terminus of human TDP43. The immunogen is located within amino acids 60 - 110 of TDP43.

DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It 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. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.

TDP43 antibody can be stored at 4°C for three months and -20°C, stable for up to one year. Avoid repeated freeze-thaw cycles. Antibodies should not be exposed to prolonged high temperatures.