|Other Names||TAR DNA-binding protein 43, TDP-43, TARDBP, TDP43|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP13763a was selected from the N-term region of TARDBP. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. 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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HIV-1, the causative agent of acquired immunodeficiencysyndrome (AIDS), contains an RNA genome that produces achromosomally integrated DNA during the replicative cycle.Activation of HIV-1 gene expression by the transactivator Tat isdependent on an RNA regulatory element (TAR) located downstream ofthe transcription initiation site. The protein encoded by this geneis a transcriptional repressor that binds to chromosomallyintegrated TAR DNA and represses HIV-1 transcription. In addition,this protein regulates alternate splicing of the CFTR gene. Asimilar pseudogene is present on chromosome 20. [provided byRefSeq].
Kim, S.H., et al. J. Biol. Chem. 285(44):34097-34105(2010)Geser, F., et al. Arch. Neurol. 67(10):1238-1250(2010)Mackenzie, I.R., et al. Lancet Neurol 9(10):995-1007(2010)Shan, X., et al. Proc. Natl. Acad. Sci. U.S.A. 107(37):16325-16330(2010)McKee, A.C., et al. J. Neuropathol. Exp. Neurol. 69(9):918-929(2010)
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