The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesICC/IF: Use at a concentration of 1 µg/ml.
WB: Use at a concentration of 1 µg/ml. Detects a band of approximately 50 kDa (predicted molecular weight: 45 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionDNA 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.
Tissue specificityUbiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
Involvement in diseaseDefects in TARDBP are the cause of amyotrophic lateral sclerosis type 10 (ALS10) [MIM:612069]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of ALS is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
DomainThe RRM domains can bind to both DNA and RNA.
Post-translational modificationsHyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Ubiquitinated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Cleaved to generate C-terminal fragments in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Cellular localizationNucleus. 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.
ICC/IF image of ab42474 stained human HeLa cells. The cells were 4% PFA fixed (10 min), permabilised in 0.1% PBS-Tween (20 min) and incubated with the antibody (ab42474, 1µg/ml) for 1h at room temperature. 1%BSA / 10% normal goat serum / 0.3M glycine was used to block non-specific protein-protein interactions. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red). DAPI was used to stain the cell nuclei (blue). This antibody also gave a positive IF result in HEK 293, HepG2 and MCF7 cells.
Western blot - Anti-TDP43 antibody (ab42474)
All lanes : Anti-TDP43 antibody (ab42474) at 1 µg/ml
Lane 1 : Human brain tissue lysate - total protein (ab29466) Lane 2 : Human testis tissue lysate - total protein (ab30257) Lane 3 : Human spinal cord tissue lysate - total protein (ab29188) Lane 4 : Brain (Mouse) Tissue Lysate Lane 5 : Testis (Mouse) Tissue Lysate - normal tissue Lane 6 : Spinal Cord (Mouse) Tissue Lysate Lane 7 : Brain (Rat) Tissue Lysate - normal tissue Lane 8 : Rat DRG
Lysates/proteins at 10 µg per lane.
Secondary Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution