Overview

  • Product name
    Recombinant Human TSEN54 protein
  • Protein length
    Protein fragment

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      LQTTHLPDGGVRLLEKSGGLEIIFDVYQADAVATFRKNNPGKPYARMCIS GFDEPVPDLCSLKRLSYQSGDVPLIFALVDHGDISFYSFRDFTLPQDVG
    • Amino acids
      427 to 525
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab166155 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

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

Images

  • ab166155 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab166155 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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