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Products:Microbiology >> Organism >> Virus >> RNA Virus >> ssRNA positive strand virus >> HIV
Overview
Product name
TARDBP protein (Human)
See all TARDBP products (4) ...
Protein description
Recombinant fragment (aa 1-259) human TARDBP protein.
Expression host
E. coli
Properties
Purification notes
Purified.
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage buffer
Preservative: None
Constituents: 20% Glycerol, 50mM Tris acetate, 1mM EDTA, pH 7.5
Concentration
Concentration information loading...
Research areas
Epigenetics and Nuclear Signaling >> Transcription >> Other factors
Neuroscience >> Neurology process >> Neurodegenerative disease >> Other
Epigenetics and Nuclear Signaling >> DNA / RNA >> RNA Processing >> Splicing
Neuroscience >> Neurology process >> Neurodegenerative disease >> Amyotrophic lateral sclerosis
Microbiology >> Organism >> Virus >> RNA Virus >> ssRNA positive strand virus >> HIV
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Western blot - TARDBP protein (Human) (ab75468)
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Applications
Show applications key
Our Abpromise guarantee covers the use of ab75468 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: Use at an assay dependent dilution.
ELISA: Use at an assay dependent dilution.
Inhib: Use at an assay dependent dilution.
Protein info
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.
Tissue specificity
Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
Involvement in disease
Defects in TARDBP are the cause of amyotrophic lateral sclerosis type 10 (ALS10) [MIM:612069]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of ALS is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
Sequence similarities
Contains 2 RRM (RNA recognition motif) domains.
Domain
The RRM domains can bind to both DNA and RNA.
Post-translational
modifications
Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Ubiquitinated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Cleaved to generate C-terminal fragments in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Cellular localization
Nucleus. 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.
Target information above from: UniProt accessionQ13148
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
TARDBP protein (Human) images:
References for TARDBP protein (Human) (ab75468)
ab75468 has not yet been referenced specifically in any publications.
Publishing research using ab75468? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"