Rabbit anti-Human, rat TDP43 Polyclonal Antibody | anti-TARDBP antibody

Anti-TDP43-NEW!

Cat. Num. AAA502159

• Gene Names: TARDBP; ALS10; TDP-43
• Reactivity: Human, rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Affinity Purified
• Synonyms: TDP43; Polyclonal Antibody; Anti-TDP43-NEW!; anti-TARDBP antibody

• Host: Rabbit
• Reactivity: Human, rat
• Clonality: Polyclonal
• Specificity: Rat, Human; Antibody Specificity: Specific for the ~ 43kDa TDP-43 protein in Western blots of rat brain lysate. Expected molecular weight is dependent upon the TDP-43 species present in sample (full-length vs. truncated TDP-43). Under non-denaturing conditions (for example, by IHC), this antibody detects TDP-43 inclusions in human brain tissue with TDP-43 proteinopathy, but does not detect full-length nuclear TDP-43. Under denaturing conditions (for example, by Western Blot analysis) this antibody detects the C-terminus of full-length and truncated human TDP-43.
• Purity/Purification: Affinity Purified
• Form/Format: Liquid in Phosphate Buffer containing 30% glycerol.

• Applicable Applications for anti-TARDBP antibody: Western Blot, Immunohistochemistry
• Application Notes: WB: 1:1,000; IHC: 1:250
• Antigen: Synthetic peptide C-terminal to the caspase-cleavage site (between D219 and V220) of human TDP-43.
• Immunogen Species: Human
• Species Reactivity Note: The antibody has been directly tested for reactivity in human and rat.
• Biological Significance: TDP43 (Tar DNA Binding 43, TARDBP) was originally identified as a protein which binds to the "transactivation response" (TAR) sequence found in the long terminal repeat of the HIV-1 virus genome (Ou et al.,1995). UV cross-linking of HeLa cell extract revealed a 43kDa protein which was cloned and sequenced and shown to contain two copies of the ~90 amino acid RRM domain. RRM is an acronym for RNA Recognition Motif, and this domain is found in many proteins which bind single stranded RNA and some which bind single stranded DNA. Northern blots showed that the protein is ubiquitous in tissue expression. Much interest has been focused on TDP43 recently due to its association with the inclusions seen in frontotemporal lobar degeneration and Amyotrophic Lateral Sclerosis (Neumann et al., 2006). The protein is present in these inclusions in partially degraded, hyperphosphorylated and ubiquitinated forms.
• Quality Control Test: Western blots performed on each lot.
• Preparation and Storage: Store at -20 degree C; stable for at least one year.

Testing Data

References

Ou SH, Wu F, Harrich D, García-Martínez LF and Gaynor RB. (1995) Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs. J Virol. 69:3584-96.
Neumann, M.; Sampathu, D. M.; Kwong, L. K.; Truax, A. C.; Micsenyi, M. C.; Chou, T. T.; Bruce, J.; Schuck, T.; Grossman, M.; Clark, C. M.; McCluskey, L. F.; Miller, B. L.; Masliah, E.; Mackenzie, I. R.; Feldman, H.; Feiden, W.; Kretzschmar, H. A.; Trojanowski, J. Q.; Lee, V. M.-Y. (2006). Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130-133.
Forman MS, Trojanowski JQ and Lee VM-Y. (2007). TDP-43: a novel neurodegenerative proteinopathy. Current Opinions in Neurobiology 17:548-55.

NCBI and Uniprot Product Information

• NCBI GI #: 6678271
• NCBI GeneID: 23435
• NCBI Accession #: NP_031401.1
• NCBI GenBank Nucleotide #: NM_007375.3
• UniProt Accession #: Q13148; Q53H27; Q6FI92; Q96DJ0; A4GUK4; A4GUK5; A4GUK6; B2R629; E2PU12; Q9H256
• Molecular Weight: 43 kDa
• NCBI Official Full Name: TAR DNA-binding protein 43
• NCBI Official Synonym Full Names: TAR DNA binding protein
• NCBI Official Symbol: TARDBP
• NCBI Official Synonym Symbols: ALS10; TDP-43
• NCBI Protein Information: TAR DNA-binding protein 43; OTTHUMP00000002171; OTTHUMP00000002172; OTTHUMP00000002173; TAR DNA-binding protein-43
• UniProt Protein Name: TAR DNA-binding protein 43
• Protein Family: TAR DNA-binding protein
• UniProt Gene Name: TARDBP
• UniProt Synonym Gene Names: TDP43
• UniProt Entry Name: TADBP_HUMAN

NCBI Description

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]

Uniprot Description

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. Ref.2 Ref.12

Subunit structure: Homodimer

By similarity. Binds specifically to pyrimidine-rich motifs of TAR DNA and to single stranded TG repeated sequences. Binds to RNA, specifically to UG repeated sequences with a minimun of six contiguous repeats. Interacts with ATNX2; the interaction is RNA-dependent. Ref.15

Subcellular location: Nucleus. Note: 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. Ref.2 Ref.11

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

Domain: The RRM domains can bind to both DNA and RNA

Post-translational modification: Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Ref.11 Ref.14Ubiquitinated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Ref.2 Ref.11Cleaved to generate C-terminal fragments in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.

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. Ref.15 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29

Sequence similarities: Contains 2 RRM (RNA recognition motif) domains.