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Rabbit Anti-TARDBP Recombinant Antibody (BA0293) (CBMAB-0862CQ)


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Summary

Host Animal
Rabbit
Specificity
Mouse, Rat, Human
Clone
BA0293
Antibody Isotype
IgG
Application
WB, IHC-P, IF

Basic Information

Immunogen
Synthetic peptide
Specificity
Mouse, Rat, Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
TARDBP Gene(Protein Coding) TAR DNA Binding Protein
Introduction
HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. 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.
Entrez Gene ID
Human23435
Mouse230908
Rat298648
UniProt ID
HumanQ13148
MouseQ921F2
RatI6L9G6
Alternative Names
ALS10; TDP-43
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).
Biological Process
3'-UTR-mediated mRNA destabilizationManual Assertion Based On ExperimentIDA:CACAO
3'-UTR-mediated mRNA stabilizationManual Assertion Based On ExperimentIDA:BHF-UCL
mRNA processingIEA:UniProtKB-KW
Negative regulation by host of viral transcriptionManual Assertion Based On ExperimentIDA:BHF-UCL
Negative regulation of gene expressionManual Assertion Based On ExperimentIMP:CACAO
Negative regulation of protein phosphorylationManual Assertion Based On ExperimentIMP:CACAO
Nuclear inner membrane organizationManual Assertion Based On ExperimentIMP:CACAO
Positive regulation of protein import into nucleusIEA:Ensembl
Regulation of apoptotic processManual Assertion Based On ExperimentIMP:CACAO
Regulation of cell cycleManual Assertion Based On ExperimentIMP:CACAO
Regulation of circadian rhythmIEA:Ensembl
Regulation of gene expressionManual Assertion Based On ExperimentIBA:GO_Central
Regulation of protein stabilityManual Assertion Based On ExperimentIMP:UniProtKB
Rhythmic processIEA:UniProtKB-KW
RNA splicingManual Assertion Based On ExperimentIDA:BHF-UCL
Cellular Location
Nucleus
Cytoplasm
Cytoplasm, Stress granule
Mitochondrion
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).
Involvement in disease
Amyotrophic lateral sclerosis 10 (ALS10):
A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
PTM
Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Phosphorylated upon cellular stress.
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.
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For research use only. Not intended for any clinical use.

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