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TDP-43 / TARDBP Antibody (clone 2E2-D3)
Mouse Monoclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC-P, IF, E, RNAi |
|---|---|
| Primary Accession | Q13148 |
| Reactivity | Human |
| Host | Mouse |
| Clonality | Monoclonal |
| Clone Names | 2E2-D3 |
| Calculated MW | 45kDa |
| Dilution | IF (10 µg/ml), IHC-P (5 µg/ml), |
| Gene ID | 23435 |
|---|---|
| Other Names | TAR DNA-binding protein 43, TDP-43, TARDBP, TDP43 |
| Reconstitution & Storage | Store at -20°C. Aliquot to avoid freeze/thaw cycles. |
| Precautions | TDP-43 / TARDBP Antibody (clone 2E2-D3) 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). Regulates also 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:23398327, PubMed:19765185). Participates also 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 |
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Background
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.
References
Ou S.-H.I.,et al.J. Virol. 69:3584-3596(1995).
Strong M.J.,et al.Mol. Cell. Neurosci. 35:320-327(2007).
Ota T.,et al.Nat. Genet. 36:40-45(2004).
Ebert L.,et al.Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases.
Suzuki Y.,et al.Submitted (APR-2005) to the EMBL/GenBank/DDBJ databases.
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