Mouse Anti-TARDBP Monoclonal Antibody (3H8) (CBMAB-1631-YC)

TARDBP Gene(Protein Coding) TAR DNA Binding Protein

TARDBP 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.

ALS10; TDP-43

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).

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

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).

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.

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.