Overview

  • Product name
    Recombinant Human ETHE1 protein
  • Protein length
    Full length protein

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHH SSGLVPRGSH MGSHMLSQRG GSGAPILLRQ MFEPVSCTFT YLLGDRESRE AVLIDPVLET APRDAQLIKE LGLRLLYAVN THCHADHITG SGLLRSLLPG CQSVISRLSG AQADLHIEDG DSIRFGRFAL ETRASPGHTP GCVTFVLNDH SMAFTGDALL IRGCGRTDFQ QGCAKTLYHS VHEKIFTLPG DCLIYPAHDY HGFTVSTVEE ERTLNPRLTL SCEEFVKIMG NLNLPKPQQI DFAVPANMRC GVQTPTA
    • Molecular weight
      29 kDa including tags
    • Amino acids
      13 to 254
    • Tags
      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab119454 in the following tested applications.

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

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • Mass spectrometry
    MALDI-TOF
  • Purity
    > 90 % 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: 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride

General Info

  • Alternative names
    • Ethe1
    • ETHE1 protein, mitochondrial precursor
    • ETHE1_HUMAN
    • ethylmalonic encephalopathy 1
    • Ethylmalonic encephalopathy protein 1
    • hepatoma subtracted clone one
    • Hepatoma subtracted clone one protein
    • HSCO
    • mitochondrial
    • Protein ETHE1
    • Protein ETHE1 mitochondrial
    • YF13H12
    see all
  • Function
    Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA.
  • Tissue specificity
    Ubiquitously expressed.
  • Involvement in disease
    Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) [MIM:602473]. EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.
  • Sequence similarities
    Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.
  • Cellular localization
    Cytoplasm. Nucleus. Mitochondrion matrix. According to PubMed:12398897, it is cytoplasmic and nuclear. According to PubMed:14732903, it is found in the mitochondrial matrix.
  • Information by UniProt

Images

  • 15% SDS-PAGE analysis of ab119454 (3µg)

References

ab119454 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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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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