Overview

  • Product name

    Anti-TSEN54 antibody [EPR10062]
    See all TSEN54 primary antibodies
  • Description

    Rabbit monoclonal [EPR10062] to TSEN54
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IF, IHC-Pmore details
    Unsuitable for: Flow Cyt or IP
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human TSEN54 aa 1-100 (Cysteine residue). The exact sequence is proprietary.
    Database link: Q7Z6J9

  • Positive control

    • MCF7, 293T and Y79 cell lysates; Human pancreas tissue; MCF7 cells.
  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab178696 in the following tested applications.

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

Application Abreviews Notes
WB 1/1000 - 1/10000. Predicted molecular weight: 59 kDa.
ICC/IF 1/100 - 1/250.
IHC-P 1/50 - 1/100.
  • Application notes
    Is unsuitable for Flow Cyt or IP.
  • Target

    • Function

      Non-catalytic subunit of the tRNA-splicing endonuclease complex, a complex responsible for identification and cleavage of the splice sites in pre-tRNA. It cleaves pre-tRNA at the 5' and 3' splice sites to release the intron. The products are an intron and two tRNA half-molecules bearing 2',3' cyclic phosphate and 5'-OH termini. There are no conserved sequences at the splice sites, but the intron is invariably located at the same site in the gene, placing the splice sites an invariant distance from the constant structural features of the tRNA body. The tRNA splicing endonuclease is also involved in mRNA processing via its association with pre-mRNA 3' end processing factors, establishing a link between pre-tRNA splicing and pre-mRNA 3' end formation, suggesting that the endonuclease subunits function in multiple RNA-processing events.
    • Involvement in disease

      Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 4 (PCH4) [MIM:225753]. Pontocerebellar hypoplasia (PCH) is a heterogeneous group of disorders characterized by an abnormally small cerebellum and brainstem. PCH4 is characterized by severe course and early lethality.
      Defects in TSEN54 are the cause of pontocerebellar hypoplasia type 2A (PCH2A) [MIM:277470]. Pontocerebellar hypoplasia (PCH) is a heterogeneous group of disorders characterized by an abnormally small cerebellum and brainstem. PCH type 2 is characterized by progressive microcephaly from birth combined with extrapyramidal dyskinesia and chorea, epilepsy, and normal spinal cord findings.
    • Sequence similarities

      Belongs to the SEN54 family.
    • Post-translational
      modifications

      Phosphorylated upon DNA damage, probably by ATM or ATR.
    • Cellular localization

      Nucleus. Nucleus > nucleolus. May be transiently localized in the nucleolus.
    • Information by UniProt
    • Database links

    • Alternative names

      • HsSen54 antibody
      • PCH2A antibody
      • PCH4 antibody
      • sen54 antibody
      • SEN54 homolog antibody
      • SEN54_HUMAN antibody
      • SEN54L antibody
      • tRNA-intron endonuclease SEN54 antibody
      • tRNA-splicing endonuclease subunit SEN54 antibody
      • Tsen54 antibody
      • TSEN54 tRNA splicing endonuclease subunit antibody
      see all

    Images

    • All lanes : Anti-TSEN54 antibody [EPR10062] (ab178696) at 1/1000 dilution

      Lane 1 : MCF7 cell lysate
      Lane 2 : 293T cell lysate
      Lane 3 : Y79 cell lysate

      Lysates/proteins at 10 µg per lane.

      Predicted band size: 59 kDa

    • Immunofluorescent analysis of MCF7 cells labeling TSEN54 with ab178696 at 1/100 dilution.

    • Immunohistochemical analysis of paraffin-embedded Human pancreas tissue labeling TSEN54 with ab178696 at 1/50 dilution.

    References

    This product has been referenced in:

    • Breuss MW  et al. Autosomal-Recessive Mutations in the tRNA Splicing Endonuclease Subunit TSEN15 Cause Pontocerebellar Hypoplasia and Progressive Microcephaly. Am J Hum Genet 99:228-35 (2016). Read more (PubMed: 27392077) »
    See 1 Publication for this product

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