Target：TADBP

Fields：mRNA surveillance pathway;Amyotrophic lateral sclerosis;Pathways of neurodegeneration - multiple diseases

Gene Name：TARDBP TDP43

Protein Name：TAR DNA-binding protein 43 (TDP-43)

Human Gene Id：23435

Human Swiss Prot No：Q13148

Mouse Swiss Prot No：Q921F2

Immunogen：Synthesized peptide derived from human protein . at AA range: 41-90

Specificity：TADBP Polyclonal Antibody detects endogenous levels of protein.

Formulation：Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000 ELISA 1:5000-20000

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD)：45kD

Background：TAR DNA binding protein(TARDBP) Homo sapiens 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. [provided by RefSeq, Jul 2008],

Function：disease:Defects in TARDBP are a 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. TARDBP is the primary component of ubiquitin-positive inclusion bodies found in ALS and in frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLDU).,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 o

Subcellular 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). .

Expression：Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.