immunohistochemistry: 5-10 μg/mL using rat, mouse, and human kidney indirect immunofluorescence: 5-10 μg/mL using human HepG2 cells western blot: 1-2 μg/mL using human U2OS cell lysates
TDP-43 (TAR DNA binding protein, TARDP) is a 414 amino acid nuclear protein and is a member of the heterogenous nuclear ribonucleoproteins (hnRNPs) family that bind single stranded RNA. It is encoded by the gene mapped to human chromosome 1p36.22. The encoded protein belongs to the family of heterogenous nuclear ribonucleoproteins (hnRNPs) that bind single stranded RNA. TDP-43 is ubiquitously expressed and is characterized with two RNA-recognition motifs and a glycine-rich C-terminal region.
Specificity
Anti-TDP-43 (C-terminal) specifically recognizes human, mouse, and rat TDP-43.
Biochem/physiol Actions
Heterogeneous nuclear ribonucleoproteins (hnRNPs) play a vital role in generation and processing of RNA, including transcription, splicing, transport and stability. TDP-43 acts as a transcription regulator for human immunodeficiency virus (HIV). Abnormal phosphorylation of TDP-43 at Ser409/410 contributes to the pathology of frontotemporal lobe degeneration subtype (FTLD-U) and amyotrophic lateral sclerosis (ALS).
Physical form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
Storage and Stability
Store at –20 °C. For continuous use, the product may be stored at 2–8 °C for up to one month. For extended storage, freeze in working aliquots at –20 °C. Repeated freezing and thawing, or storage in “frost-free” freezers, is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.
Disclaimer
Unless otherwise stated in our catalog, our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
TAR-DNA binding protein 43 (TDP-43) proteinopathy is a common brain pathology in elderly persons, but much remains to be learned about this high-morbidity condition. Published stage-based systems for operationalizing disease severity rely on the involvement (presence/absence) of pathology in specific
Loss-of-function mutations in TANK-binding kinase 1 cause genetic amyotrophic lateral sclerosis and frontotemporal dementia. Consistent with incomplete penetrance in humans, haploinsufficiency of TANK-binding kinase 1 did not cause motor symptoms in mice up to 7 months of age in a previous
Journal of neuroscience research, 94(7), 671-682 (2016-03-21)
TAR DNA binding protein 43 (TDP-43) is a nuclear protein that has been shown to have altered homeostasis in the form of neuronal nuclear and cytoplasmic aggregates in some familial and almost all cases of sporadic amyotrophic lateral sclerosis as
The role of TDP-43 in amyotrophic lateral sclerosis and frontotemporal dementia
Mackenzie, Ian RA and Rademakers, Rosa
Current Opinion in Neurology, 21(6), 693-693 (2008)
TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression
Ayala YM, et al.
Proceedings of the National Academy of Sciences, 105(10), 3785-3789 (2008)
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