|Application ||WB, E|
|Other Accession||NP_031401, 23435, 230908 (mouse)|
|Predicted||Rat, Pig, Dog, Cow|
|Calculated MW||44740 Da|
|Other Names||TAR DNA-binding protein 43, TDP-43, TARDBP, TDP43|
|Format||0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||Goat Anti-TDP-43 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.
HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. Activation of HIV-1 gene expression by the transactivator Tat is dependent on an RNA regulatory element (TAR) located downstream of the transcription initiation site. The protein encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. A similar pseudogene is present on chromosome 20.
TARDBP gene mutations among Chinese patients with sporadic amyotrophic lateral sclerosis. Huang R, et al. Neurobiol Aging, 2010 Aug 12. PMID 20708823.
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. Xu YF, et al. J Neurosci, 2010 Aug 11. PMID 20702714.
Amyotrophic lateral sclerosis-frontotemporal lobar dementia in 3 families with p.Ala382Thr TARDBP mutations. Chi貌 A, et al. Arch Neurol, 2010 Aug. PMID 20697052.
Large-scale screening of TARDBP mutation in amyotrophic lateral sclerosis in Japanese. Iida A, et al. Neurobiol Aging, 2010 Jul 30. PMID 20675015.
TARDBP Mutations in Frontotemporal Lobar Degeneration: Frequency, Clinical Features, and Disease Course. Borroni B, et al. Rejuvenation Res, 2010 Jul 20. PMID 20645878.
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