|Application ||WB, IF, ICC, E|
|Other Accession||ABO32290, 130750552|
|Reactivity||Human, Mouse, Rat|
|Calculated MW||44740 Da|
|Application Notes||TDP43 antibody can be used for detection of TDP43 by Western blot at 0.5 - 2 µg/mL. Antibody can also be used for immunocytochemistry starting at 5 µg/mL. For immunofluorescence start at 20 µg/mL.|
|Target/Specificity||TARDBP; At least two isoforms are known to exist for this protein; this TDP43 antibody only recongizes the longer of the two isoforms.|
|Reconstitution & Storage||TDP43 antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.|
|Precautions||TDP43 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||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.|
|Cellular Location||Nucleus. Note=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|
|Tissue Location||Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen|
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Provided below are standard protocols that you may find useful for product applications.
TDP43 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.
Ou SH, Wu F, Garcia-Martinez LF, et al. Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs. J. Virol.1995; 69:3584-96.
Buratti E, Dork T, Zuccato E, et al. Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping. EMBO J.2001; 20:1774-84.
Neumann M, Sampathu DM, Kwong LK, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science2006; 314:42-3.
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