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Recombinant Human Protein ETHE1, mitochondrial (ETHE1)

ACP03431

Number
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Specifications


Cat.No ACP03431 Target NameETHE1
FormLiquid or Lyophilized powderExpression SystemE.coli
Expression Range8-254aaMol Weight43.1kDa
Protein LengthFull Length of Mature ProteinPurityGreater than 90% as determined by SDS-PAGE.
Storage Buffer5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information


Target SpeciesHumanUniprot IDO95571
Background Information
  • Uniprot Id

    O95571

  • Target Species

    Human

  • Target Name

    ETHE1

  • Target Full Name

    Persulfide dioxygenase ETHE1, mitochondrial

  • Target Function

    Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, due to the inhibition of cytochrome c oxidase. First described as a protein that can shuttle between the nucleus and the cytoplasm and suppress p53-induced apoptosis by sequestering the transcription factor RELA/NFKB3 in the cytoplasm and preventing its accumulation in the nucleus.

  • Target Involvement

    Ethylmalonic encephalopathy (EE)

  • Target Subcellular Location

    Cytoplasm. Nucleus. Mitochondrion matrix.

  • Target Protein Families

    Metallo-beta-lactamase superfamily, Glyoxalase II family

  • Target Tissue Specificity

    Ubiquitously expressed.

  • Target Research Area

    Cell Biology

  • Target Synonyms

    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

  • Target Background

    This gene encodes a member of the metallo beta-lactamase family of iron-containing proteins involved in the mitochondrial sulfide oxidation pathway. The encoded protein catalyzes the oxidation of a persulfide substrate to sulfite. Certain mutations in this gene cause ethylmalonic encephalopathy, an infantile metabolic disorder affecting the brain, gastrointestinal tract and peripheral vessels. Alternative splicing results in multiple transcript variants encoding different isoforms.

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