Description

  • Product name

    Recombinant Human TDP43 protein
    See all TDP43 proteins and peptides
  • Purity

    > 85 % SDS-PAGE.
    ab224788 was purified using conventional chromatography techniques.
  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MSEYIRVTEDENDEPIEIPSEDDGTVLLSTVTAQFPGACGLRYRNPVSQC MRGVRLVEGILHAPDAGWGNLVYVVNYPKDNKRKMDETDASSAVKVKRAV QKTSDLIVLGLPWKTTEQDLKEYFSTFGEVLMVQVKKDLKTGHSKGFGFV RFTEYETQVKVMSQRHMIDGRWCDCKLPNSKQSQDEPLRSRKVFVGRCTE DMTEDELREFFSQYGDVMDVFIPKPFRAFAFVTFADDQIAQSLCGEDLII KGISVHISNAEPKHNSNRQLERSGRFGGNPGGFGNQGGFGNSRGGGAGLG NNQGSNMGGGMNFGAFSINPAMMAAAQAALQSSWGMMGMLASQQNQSGPS GNNQNQGNMQREPNQAFGSGNNSYSGSNSGAAIGWGSASNAGSGSGFNGG FGSSMDSKSSGWGM
    • Predicted molecular weight

      45 kDa
    • Amino acids

      1 to 414
    • Additional sequence information

      NP_031401.

Specifications

Our Abpromise guarantee covers the use of ab224788 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.02% DTT, 0.03% EDTA, 10% Glycerol, 0.58% Sodium chloride, 0.32% Tris HCl

General Info

  • Alternative names

    • ALS10
    • OTTHUMP00000002171
    • OTTHUMP00000002172
    • OTTHUMP00000002173
    • TADBP_HUMAN
    • TAR DNA binding protein
    • TAR DNA binding protein 43
    • TAR DNA-binding protein 43
    • TARDBP
    • TDP 43
    • TDP-43
    • TDP43
    see all
  • 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.
  • Information by UniProt

Images

  • 15% SDS-PAGE analysis of 3 µg ab224788.

     

References

ab224788 has not yet been referenced specifically in any publications.

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