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TDP43 Antibody
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- 实验流程
- 背景知识
Application 
| WB, IF, ICC, E |
|---|---|
| Primary Accession | Q13148 |
| Other Accession | ABO32290, 130750552 |
| Reactivity | Human, Mouse, Rat |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Calculated MW | 44740 Da |
| Concentration (mg/ml) | 1 mg/mL |
| Conjugate | Unconjugated |
| 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. |
| Gene ID | 23435 |
|---|---|
| Other Names | TAR DNA-binding protein 43, TDP-43, TARDBP, TDP43 |
| 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. |
| Name | TARDBP {ECO:0000303|PubMed:18396105, ECO:0000312|HGNC:HGNC:11571} |
|---|---|
| Function | RNA-binding protein that is involved in various steps of RNA biogenesis and processing (PubMed:23519609). Preferentially binds, via its two RNA recognition motifs RRM1 and RRM2, to GU-repeats on RNA molecules predominantly localized within long introns and in the 3'UTR of mRNAs (PubMed:23519609, PubMed:24240615, PubMed:24464995). In turn, regulates the splicing of many non-coding and protein-coding RNAs including proteins involved in neuronal survival, as well as mRNAs that encode proteins relevant for neurodegenerative diseases (PubMed:21358640, PubMed:29438978). Plays a role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts (PubMed:28794432). Also regulates mRNA stability by recruiting CNOT7/CAF1 deadenylase on mRNA 3'UTR leading to poly(A) tail deadenylation and thus shortening (PubMed:30520513). In response to oxidative insult, associates with stalled ribosomes localized to stress granules (SGs) and contributes to cell survival (PubMed:19765185, PubMed:23398327). Also participates in the normal skeletal muscle formation and regeneration, forming cytoplasmic myo-granules and binding mRNAs that encode sarcomeric proteins (PubMed:30464263). Plays a role in the maintenance of the circadian clock periodicity via stabilization of the CRY1 and CRY2 proteins in a FBXL3-dependent manner (PubMed:27123980). Negatively regulates the expression of CDK6 (PubMed:19760257). Regulates the expression of HDAC6, ATG7 and VCP in a PPIA/CYPA-dependent manner (PubMed:25678563). |
| Cellular Location | Nucleus. Cytoplasm. Cytoplasm, Stress granule Mitochondrion. Note=Continuously travels in and out of the nucleus (PubMed:18957508). Localizes to stress granules in response to oxidative stress (PubMed:19765185). A small subset localizes in mitochondria (PubMed:28794432). |
| Tissue Location | Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
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.
REFERENCES
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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